Assesment, treatment and prevention of atypical hemolytic uremic syndrome

Hemolytic uremic syndrome [HUS] is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: [1] D+ HUS: Presentation with a preceding diarrhea; [2] typical HUS: D+ HUS with a single and self-limited episode; [3] atypical HUS [aHUS]: Indicated those with complement dysregulation; [4] recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia [MAHA] after improvement of hematologic abnormalities; and [5] familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease [ESRD] in children. It has a high recurrence after renal transplantation [TP] in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS

[Acute disseminated encephalomyelitis]

Acute disseminated encephalomyelitis [ADEM] is an acute, inflammatory, monophasic, demyelinating and immune-mediated disorder of central nervous system; occurs mostly in children after systemic viral infections or vaccinations. Acute polysymptomatic neurologic signs such as encephalopathy, paralysis of limbs, cranial nerve involvement, ataxia and optic neuritis are common manifestations. Brain magnetic resonance imaging study is essential for diagnosis and enabling prompt diagnosis and treatment. Evidence of multifocal lesions of demyelination in subcortical white matter are seen. They are usually bilateral and asymmetrical. Treatment options have included steroids, immunoglobulins, and plasmapheresis. ADEM is treatable and prognosis is good

[Surfactant effects on mortality and morbidity of respiratory distress syndrome in neonates]

The purpose of this study was to compare the mortality and morbidity rates of neonatal respiratory distress syndrome [RDS] treated with and without surfactant in ventilated patients. In this descriptive-analytical study, the morbidity and mortality rates of RDS in 50 patients of the study group treated with surfactant were compared with 66 patients of control group treated without surfactant. The children of the control group received treatment at a time when surfactant was not available in market. Surfactant was used as Survanta during the first 24 hours of life with a dose of 4 ml/kg in four different positions through tracheal tube only once. The two groups were comparable in baseline parameters including birth weight, sex, gestational age and primary diagnosis. Mortality reduced significantly. There were no differences in the incidence of severe complications including, intraventricular hemorrhage and patent ductus arteriosus, but pneumothorax, sepsis and pulmonary hemorrhage occurred more common in the control group. Use of surfactant in neonatal respiratory distress syndrome significantly decreased mortality, pneumothorax, sepsis and pulmonary hemorrhage

Ceftriaxone induced biliary pseudolithiasis in children: report of 14 cases

Biliary pseudolithiasis has been reported in patients who received ceftriaxone therapy. In this study we evaluated children with ceftriaxone associated pseudolithiasis that was discovered incidentally in US examination. The study includes 14 children with gallstones in Ultrasound without biliary symptoms with recent ceftriaxone administration. All of them were treated for suspected or definite bacterial infection with ceftriaxone 50-100mg/kg/day divided into 2 equal intravenous doses under conditions of adequate hydration. There were no other known underlying diseases for gallstone. Fourteen patients [11 boys and 3 girls] with mean age of 4.5 years [range: 2 months to 14 years] were studied. Following cessation of treatment with ceftriaxone, a complete resolution of the lithiasis was seen in most of followed cases. All patients were free from biliary symptoms [Right upper quadrant pain, Cholestasis] during observation. Consultations with surgeon or subspecialist due to reported "gallstone in the Ultrasound" were performed in about two-thirds of patients. Development of pseudolithiasis after ceftriaxone administration is not uncommon and should be known by pediatricians and radiologists in order to avoid unnecessary surgery or additional consultations

Enoxaparin plus ticlopidine: an effective combination therapy for intracardiac thrombi in thalassemia intermedia

Patients with thalassemia intermedia have an increased risk of thrombotic events as compared to the general population. We describe two cases of thalassemia intermedia with intracardiac thrombi who failed to response to traditional anticoagulation therapy with Unfractionated Heparin and Aspirin; but thrombolysis occurred following combination therapy with Ticlopidine and Enoxaparin. Our experience in patients shown combination of Enoxaparin and Ticlopidine is effective for treatment of ventricular thrombus in TI patients

Earlobe crease and coronary artery disease

Diagonal ear lobe crease [ELC] has been introduced as a diagnostic physical sign for coronary artery disease [CAD] and the aim of this study was to evaluate the association between ELC and CAD in Iranians. In a cross- sectional study, 106 consecutive patients, referred from cardiologists for angiography, were studied for presence of ELC and CAD in four academic hospitals in Tehran. ELC categorized based on Ishii classification and CAD defined as at least 50% narrowing in one of the three major epicardial vessels. The average age in 70 men and 36 women was 50.14 +/- 14.11 years. Based on angiographic results, patients were divided in two groups. In 57 patients of CAD positive group, 34 patients had ELC and in 49 patients of CAD negative group only 13 patients had ELC. [P<0.05] After adjusting for age, ELC was a significant predictor of CAD [P<0.05]. The severity of ELC [size and unilateral or bilateral] was significantly related to presence of CAD [P<0.05]. The observed sensitivity, specificity, positive predictive value and negative predictive value of ELC for diagnosis of CAD were in the following order: 59.6%, 73.5%, 72.3% and 61%. In Iranians, the presence of ELC is associated with CAD

Excessive crying of infancy; a report of 200 cases

Excessive crying of infancy; a single continuous episode of crying more than 2 common and often serious problem for parents. The objective of this study was to determine the incidence and etiology of acute episodes of excessive infant crying and to propose an approach for taking care of them. In this cross sectional study all patients less than 2 years old presented to the Emergency Department [ED] with >/= 2 hours of crying with an unapparent cause for their parents were included in this study. Patients with fever, chronic disease and any disease 72 hours proceeding the crying were excluded. The incidence of excessive crying was 200 [1.74%] among the 13600 ED visits in 2 consecutive years .The three most common causes were colic [29.5%], acute otitis media [15.5%] and constipation [5.5%]. History and physical examination led to diagnosis in 86% of cases. Many conditions and diseases may cause excessive crying in infants. Complete history and physical examination of all parts of the body provide clues for the diagnosis in most cases and help the physician to avoid over diagnosis and over treatment

Sweat test in asthmatic children; a single center study

Asthma and Cystic Fibrosis [CF] have some identical manifestations. In the present study, the frequency of positive sweat test was assessed in asthmatic children. This cross-sectional study was performed in asthmatic children, who were referred to Bahrami Children Hospital, Tehran, during July 2003 to July 2005. Sweat test was performed for all children. One hundred and thirty five [95 males and 40 females] asthmatic were studied. Sweat test was positive in 35 [26%] patients. Major signs and symptoms among these 35 patients were: Cough [35/35], Dyspnea [7/35], Chronic Diarrhea [6/35], and steatorrhea [1/35]. Failure to thrive was found in 31 of 35 patients. The mean duration of asthma in patients with positive test was 49.3 months, which was significantly higher than 18.1 months in the group with negative test [P=0.001]. Significant relations between recurrent respiratory tract infections [P=0.029], chronic diarrhea [P=0.001], failure to thrive [P=0.0001], and positive sweat test were found. Sweat test should be recommended in asthmatic children with recurrent upper respiratory tract infections, long duration of asthma, chronic diarrhea, and failure to thrive