Infantile nephropathic cystinosis: case series and review of literature

Cystinosis is a rare metabolic disease with an autosomal recessive inheritance. It is characterized by deposition of an extraordinary amount of cystine in different organs of the body. Children with infantile nephropathic cystinosis [INC] present with failure to thrive, polyuria, polydipsia and photophobia in early infancy. They progress to chronic renal failure [CRF] between the ages 5 to 10 years. The diagnosis of cystinosis should be considered in young children with failure to thrive or CRF of unknown etiology. Cysteamine is effective in delaying the progression of this disease. Four patients with INC from two families were followed over the last few years. All of them presented with polyuria, polydipsia, failure to thrive and rickets. Laboratory findings included glucosuria, hypophosphatemia, hypokalemia, proteinuria and later on azotemia. Therapy with cysteamine showed clinical improvement when started early

Device-associated infection rates in adult and pediatric intensive care units of hospitals in Egypt. International nosocomial infection control consortium [INICC] findings

To determine the rate of device-associated healthcare-associated infections [DA-HAIs] at a respiratory intensive care unit [RICU] and in the pediatric intensive care units [PICUs] of member hospitals of the International Nosocomial Infection Control Consortium [INICC] in Egypt. A prospective cohort DA-HAI surveillance study was conducted from December 2008 to July 2010 by applying the methodology of the INICC and the definitions of the NHSN-CDC. In the RICU, 473 patients were hospitalized for 2930 d and acquired 155 DA-HAIs, with an overall rate of 32.8%. There were 52.9 DA-HAIs per 1000 ICU-days. In the PICUs, 143 patients were hospitalized for 1535 d and acquired 35 DA-HAIs, with an overall rate of 24.5%. There were 22.8 DA-HAIs per 1000 ICU-days. The central line-associated blood stream infection [CLABSI] rate was 22.5 per 1000 line-days in the RICU and 18.8 in the PICUs; the ventilator-associated pneumonia [VAP] rate was 73.4 per 1000 ventilator-days in the RICU and 31.8 in the PICUs; and the catheter-associated urinary tract infection [CAUTI] rate was 34.2 per 1000 catheter-days in the RICU. DA-HAIs in the ICUs in Egypt pose greater threats to patient safety than in industrialized countries, and infection control programs, including surveillance and guidelines, must become a priority

Impact of management strategies on the outcome of persistent pulmonary hypertension of the newborn

The authors have indicated that they have no financial relationships relevant to this article to disclose. The study aims to evaluate associated risk factors and determine the impact of management strategies on the outcome of Pulmonary Hypertension of the Newborn [PPHN]. Prospective descriptive study included 40 neonates having PPHN. All patients received conventional therapy for PPHN, in case of failure, sildenafil was added as adjuvant therapy. The study included 23 males, 17 females with mean gesfational age 37.25 +/- 2.6 weeks. PPHN male patients had significant higher systolic pulmonary artery pressure [SPAP] and higher mortality rate compared to females [7/23 [30.4%] versus 1/17 [5.9%], P=0.04]. Infants of diabetic mothers had significant higher mortality rate [P=0.003]. Components of the blood gases; PH, PCO2, HCO3 had improved dramatically after completion of the different lines of treatment [p=0.0001]. A statistical significant drop of SPAP after application of different modalities of treatment [P=0.001]. Adding sildenafil was effective in reducing SPAP below 40 mmHg and in decreasing duration of NICU stay [P=0.0001, P=0.001 respectively]. The overall mortality rate was 8/40 neonates [20%] whereas the mortality among patients who received sildenafil in addition to conventional therapy was 1/14 neonates [7.7% of those group with P=0.001]. In absence of inhaled Nitric oxide, conventional therapy is effective in the management of PPHN. Use of oral sildenafil as an adjuvant therapy is effective in reduction of pulmonary artery pressure and hospital stay

[Refeeding syndrome] in a Kuwaiti child: clinical diagnosis and management

To report a case of refeeding syndrome in a Kuwaiti child, its clinical presentation and management. A 13-month-old Kuwaiti boy presented with acute severe malnutrition in the form of marasmic kwashiorkor. On admission, blood sugar and serum electrolytes were normal but on the 3rd day he developed typical biochemical features of refeeding syndrome in the form of hyperglycemia, severe hypophosphatemia, hy-pokalemia, hypocalcemia and hypomagnesemia. The child then received treatment appropriate for refeeding syndrome in the form of lower calorie intake with gradual increase, as well as supplementation of electrolytes, thiamine and vitamins and he eventually made a safe recovery. This case showed that during rehabilitation of a malnourished child, a severe potentially lethal electrolyte disturbance [refeeding syndrome] can occur. Careful monitoring of electrolytes before and during the refeeding phase was needed and helped to detect this syndrome early. We suggest that slow and gradual calorie increase in the 'at-risk' patient can help prevent its occurrence

1[st] line antimicrobial therapy for febrile neutropenic cancer patients

Empiric antibiotic regimens used in febrile neutropenic patients often include an extended spectrum cephalosporin, but the response of therapy in Gram positive coccal bacteremia has been unsatisfactory, thus new antibiotic with better activity against Gram positive bacteria should be tested. Antipseudomonal penicillins including piperacillin are effective against many Gram positive and Gram negative organisms. The aim of this work is to compare combination therapy with piperacillin-tazobactam plus amikacin versus ceftazidime plus amikacin as first line in treatment of febrile neutropenic cancer patients. This study is a single center, prospective and randomized trial performed in pediatric branch wards, of the National Cancer Institute, Cairo University. All patients were subjected to full clinical and laboratory evaluation including microbiological study. Doses were given according to the International Antimicrobial Therapy Cooperative Group [IATCG] of the European Organization for the Research and Treatment of Cancer [EORTC]. 164 febrile neutropenic episodes were enrolled on this study. 82 patients with 105 [64%] high risk febrile granulocytopenic episodes were considered eligible. 53 were treated with piperacillin-tazobactam plus amikacin [group A], and 52 were treated with ceftazidime plus amikacin [group B]. The overall success in group A was higher than group B, yet, the difference was statistically insignificant with a p value=0.2. Time of defervecence was significantly shorter in piperacillin-tazobactam group [p=0.001]. There was no infection related mortality in this study. Side effects were encountered in 3 [5.6%] cases receiving piperacillin-tazobactam in the form of mild skin reaction. 55.7% of positive cultures yielded Gram positive organisms. Staphylococcus species were the most common organism in both groups. Both Gram positive and negative organisms showed higher sensitivity to pipercillin-tazobactam compared to ceftazidime with a significant p value=0.05. Piperacillin-tazobactam is safe and more effective than ceftazidime in febrile neutropenia in pediatric cancer patients