Background: Assessment of glomerular filtration rate (GFR) is an important tool for monitoring renal function. Regarding to particular difficulties in measuring 24 hour urinary creatinine clearance and limitations in available methods, we intended to calculate GFR by cystatin C (Cys C) based formulas and determine correlation rate of them with current methods.Methods: We studied 72 children (38 boys and 34 girls) with several renal disorders such as reflux nephropathy, nephrotic syndrome, renal malformation, obstructive uropathy and hereditary renal disease. The 24 hour urinary creatinine clearance was the gold standard method. GFR also was measured with Schwartz formula (current method in pediatrics) and Cys C-based formulas (Grubb, Simple, Hoek and Larsson). Then correlation rates of these Cys Cbased formulas with the gold standard method and Schwartz formula were determined.Findings: Using Pearson Correlation Coefficient there was positive correlation among all formulas and the standard method (R2 for Schwartz, Hoek, Larsson, Grubb and Simple formula was 0.639, 0.722, 0.705, 0.712, 0.722 respectively), and there was statistically significant correlation (P<0.001). Cys C-based formulas could predict the variance of standard method results with high power.On the other hand, these formulas had correlation with Schwarz formula by R2 0.62-0.65 (intermediate correlation). Also, using Linear Regression and calculating the constant, it revealed that Larsson, Hoek and Grubb formulas can estimate GFR amounts with no statistical difference compared with standard method; but Schwartz and Simple formulas overestimate GFR.Conclusion: This study shows that Cys C-based formlas have strong relationship with 24 hour urinary creatinine clearance. So, it seems that with using these formulas, it is possible to determine GFR in children with kidney injury, easier and with enough correctness. Thereby the physician will be able to treat the renal disease or prevent its progression in early stages and improves the prognosis. Of course, we must emphasize that on the basis of this study, Schwartz and Simple formulas overestimate GFR.

Introduction. Children with malignancy who are under treatment with nephrotoxic drugs are at risk of renal dysfunction. Due to increased life expectancy, evaluation of drug toxicity is now of higher importance than before. The aim of this study is to compare two methods of GFR measurement. Methods. An analytical study on children with malignancy undergoing chemotherapy with nephrotoxic drugs (cisplatin, carboplatin, cyclophosphamide, ifosfamind, etoposide) during 2016 and 2017 in Mofid Children Hospital was done. Demographic information, anthropometric measurements, type of malignancy, type of chemotherapy medication and also lab tests including CBC and the biochemistry indices were recorded. The GFR of each patient was calculated using Schwartz formula and DTPA scanning. The rates were compared and the difference was analyzed statistically. Results. According to Schwartz formula, 24% of patients needed dose adjustment, while based on DTPA scanning, this rate was 6%. Comparing these two measures by paired T-test, showed a significant statistical difference (P <. 05). Schwartz formula had 25. 5% more positive results of predicting the need for dose adjustment. The two measured GFRs for each person were evaluated in terms of compatibility and correlation based on Kappa statistical method, which were incompatible and had significant difference (P <. 05). Conclusion. Using evaluative methods including Schwartz formula cannot demonstrate renal dysfunctions reliability in patients taking nephrotoxic chemotherapy drugs. Eventually if the GFR measurement method overestimates patients with renal dysfunction, the patients will not be able to make adequate use of the therapeutic effects of chemotherapy with the appropriate dosage.

In this paper, an intrinsic description of the space H(D) and its topology is presented.

Background: Glomerular filtration rate (GFR) is the best indicator to assess renal function; however, it is difficult to perform it, especially in neonates. Serum creatinine is the most commonly used marker of GFR; nevertheless, it has some limitations since it can be affected by factors other than renal function. Cystatin C, another endogenous marker used to estimate GFR, is not affected by non-renal factors. The results of some studies suggest that serum cystatin C levels are more accurate tests of kidney function than serum creatinine levels. This study aimed to estimate GFR with cystatin C-based formulas among neonates and determine the correlations between these methods and the Schwartz formula. Methods: The population of this research consisted of 99 neonates whose serum creatinine and cystatin C levels were measured concurrently. Moreover, the glomerular filtration rate was estimated using the Schwartz formula and 14 cystatin C-based formulas separately. Results: Based on the findings, all GFR values based on cystatin C formulas correlated significantly with each other (P<0. 05); however, with one exception, none of these values correlated with Schwartz GFR (P>0. 05). The only cystatin C formula that yielded values correlating with the Schwartz formula was CysCrEq, which used serum cystatin C and creatinine concomitantly. Conclusion: It can be concluded that since all GFR values based on cystatin C correlated significantly and cystatin Cwasindependent of non-renal factors, cystatin C reflected the real GFR more accurately than serum creatinine. Nonetheless, further studies with gold standard techniques are required to verify the usefulness of cystatin C-based formulas.

Schwartz Jampel syndrome or chondrodystrophic myotonia is an autosomal recessive syndrome, that is presented with stiffness, short stature, muscular hypertrophy and severe voluntary and percussion myotonia.In this report a 6 y/o boy from Tabriz is Presented who developed slow movement and gait distrubances since infancy. On exam he had facial dysmorphism, low set ears, thightness in achille and hamstrings and muscle stiffness. In electrodiagnostic study, motor and sensory NCS was within the normal range, and RST was normal. On needle EMG examination continuous, spontaneous high frequency electrical discharges (neuromyotonia) were observed in some muscles of lower limbs and face. Mild improvement was seen with carbamazepin.

We describe the multiplier algebra of the non-commutative Schwartz space. This multiplier algebra can be seen as the largest *-algebra of unbounded operators on a separable Hilbert space with the classical Schwartz space of rapidly decreasing functions as the domain. We show in particular that it is neither a Q-algebra nor mm-convex. On the other hand, we prove that classical tools of functional analysis, for example, the closed graph theorem, the open mapping theorem, or the uniform bounded-ness principle, are still available.

Introduction: Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disease characterized by muscle weakness and stiffness, abnormal bone development, short stature, joint contractures, and facial dysmorphisms. Myopathy, anatomical deformities, and malignant hyperthermia are challenging for anesthesiologists. Case Presentation: This case report describes one case of SJS. The female patient was scheduled for Blepharoplasty in Rasuol Akram General Hospital. Conclusions: These patients may have difficult intubation and be prone to malignant hyperthermia. We managed this patient by applying Rocuronium, propofol, and C-MAC video laryngoscopy.

Schwartz-Jampel syndrome, (SJS) is a rare disorder characterized by myotonia, joint contracture, facial dysmorphism and growth retardation, we present three siblings (two sisters and one brother) 19,24 and 27 years old from consanguineous healthy parents with SJS. Their clinical features were similar to those previously described. Motor and sensory nerve conduction study (NCS) were compatible with a sensorimotor polyneuro pathy. Myotonic discharges, complex repetitive discharges, myokymic discharges, positive sharp waves and fibrillation potentials were seen on EMG needle examination and MUPs were prominently neurogenic. One of the sisters had mental retardation and hypothyroidism from infancy. Thus, this is the first known report of sensorimotor polyneuropath and hypothyroidism in SJS and the first reported family with SJS from Iran.