Natural history of symptomatically treated children with cystinosis

Cystinosis is an autosomal recessive disorder of lysosomal transport of cystine. Nephropathic [infantile] cystinosis is the most common and the most severe clinical expression of the disease. To highlight the natural history of symptomatically treated children with cystinosis. A retrospective study was done on cystinotic patients who were diagnosed and treated symptomatically in children welfare teaching hospital in Baghdad from period Jun 2002 -July 2011. Twenty nine patients, 19 [66%] males and 10[34%] females who were diagnosed as cystinosis and treated symptomatically were included in the study; their ages ranged between [0.6 -12] years median 4 years. The age of onset of symptoms of tubular dysfunction ranged from [0.3-3.5 yrs] median [1.1 yrs.], positive family history of cystinosis was found in 11[37.9%] cases and parents were consanguineous in 25 [86.2%] families. All patients presented with history of polyuria, polydypsia, anorexia, vomiting, constipation and failure to thrive. Rickets was found in 22[75%] and photophobia was found in 19 [65.5%] patients. Follow up data showed renal insufficiency in eighteen patients [62%] at a median age of 9[4-12] years, hypothyroidism diagnosed in one patient [3.44%] at the age of 11 years, six [20.68%] patients died, three [10.3%] patients secondary to renal failure. Cystinosis causes extensive morbidity and death in childhood and because of high rates of consanguineous marriages in our society, we encourage a high index of suspicion in infants presenting with fluid and electrolyte loss aiming at early diagnosis and treatment of cystinosis. Leukocyte cystine levels is still needed to be available to confirm diagnosis in infants who have negative ocular examination .and to win in the fight against this terrible disease cysteamine treatment need to be available for these patients

Acute renal failure in neonates

Acute renal failure [ARF] is a common problem in admitted neonates in intensive care units. In most patients ARF accompanies with a predisposing factor such as sepsis, heart failure, perinatal asphyxia or prematurity. This study was to determine the causes and outcome of ARF in hospitalized newborn. In a descriptive cross sectional study we evaluated neonates with ARF who had been hospitalized in Children welfare teaching Hospital from July 2009 to july2010. There were 50 cases diagnosed as ARF in 2500 hospitalized neonates [2%].The male to female ratio in patients with ARF was 3.16:1. Most of involved patients were term 35[70%]. The prevalence of renal, pre renal and post renal causes of ARF was 29 [58%], 15[30%] and 6[12%] respectively. The most common predisposing factors for ARF in our study was sepsis 28[56%], genitorenal anomalies 15[30%], perinatal asphyxia 3[6%], drug toxicity in 3[6%]. One patient [2%] had respiratory distress syndrome. Among admitted neonates with ARF 6[12%] died and it was significantly higher in patients with sepsis [P<0.05]. ARF is a common emergency entity, physicians play a critical role in recognizing early ARF, preventing iatrogenic injury, and reversing the course of ARF

Chronic renal failure in children admitted to children welfare teaching hospital

Chroic renal failure [CRF] is a devastating medical, psychological, social and economic problem for patients and their families. The aim of the present study was to determine the etiology, clinical presentations and highlight treatment modalities used for patients with CRF admitted to children welfare teaching hospital. A Retrospective study of all children with CRF admitted to children welfare teaching hospital during the period from 1[st] of jan.2002 to 1[st] of jan.2007 were included in the study .CRF was defined as having glomerular filtration rate less than 80ml /min/1.73m2. The study group included 50 patients with CRF below 17 years of age, 29[58%] males and 21[42%] females. Male: female ratio 1.38:1. Their age ranged between [1m -17 year].Twenty [40%] patients were above 10 years of age. In this study the mean glomerular Filtration rate was [29.5 + 18.5ml /min/1.73m2]. Congenital abnormalities were the major cause of CRF, it was found in 18 patients [36%], followed by hereditary conditions in 14 [28%] patients and glomerular diseases in 13 [26%] patients. The most common presenting symptom was anemia. It was found in 16[32%] patients followed by hypertension in 12 patients [24%] and failure to thrive in 12[24%] patients. Twenty-one patients [42%] received peritoneal dialysis, four [8%] received hemodialysis. Renal transplant was done to 3 patients [6%]. Congenital abnormalities was to the most common cause of CRF in our patients. Establishing registry system that provide detailed information concerning the incidence, causes, and overall outcomes of mild to severe renal functional impairment acquired during developmental age can clarify further the natural history of the disease and the factors that influence its course

Causes of vomiting in hospitalized children

Vomiting is a very common symptom of disease in childhood, it occurs both in gastrointestinal and non-gastrointestinal diseases. Our aim of the study was to find out the causes of vomiting in different age groups, the improper management of vomiting in daily practice and to emphasise the usefulness of imaging study in the diagnosis. A prospective study was carried out on patients presenting with vomiting admitted to Children Welfare Teaching Hospital from the first of Dec. 2001 to the end of April 2002 .The age ranged between early neonatal periods to 12 years of age. A total of 100 child were included in this study, [65] male [35] female, male to female ratio was 1.86:1. Surgical causes of the gastrointestinal tract was the most common cause of vomiting [50%] in the neonatal period, while infectious cause formed 78.3% and 48.4% during infancy and childhood respectively. Imaging studies were carried out on 54 patients and helped to reach a final diagnosis in 21 [38.9%] patients. In this study green color vomiting was found to relate mainly [64.7%] to the intestinal obstruction with a [p] value >0.05. There was no benefit of antiemetic drugs in the treatment of vomiting, Imaging studies were helpful in reaching the correct diagnosis of vomiting in some cases, green color vomiting should make pediatrician suspicious of intestinal obstruction

Hypertension in children with nephrotic syndrome

Hypertension occurs in some patients with primary nephritic syndrome it may be mediated by changes in vasopressin activities or intravascular volume.Is to find out how many patients with idiopathic nephritic syndrome hare hypertension. The study included 50 children [29 males and 21 females] with age ranger between 1-13 years. Sixteen patients [32%] were with initial attack of nephritic syndrome, while thirty four patients [68%] presented with relapse.Nine [18%] patients had hypertension, eight [16%] patients were relapse cases, and one [2%] patient was with initial attack. Hypertension was found in four [8%] patients with steroid resistant neurotic syndrome, three [6%] patients with steroid sensitive nephritic syndrome in relapse state and two [4%] patients with steroid dependent nephritic syndrome. Abnormal renal function was found in four patients [8%], two [4%] with hypertension and two [4%] without hypertension.It is concluded the necessity of the follow up and periodical measurement of blood pressure of nephritic patients to allow early recognition and management of hypertension.The necessity of the follow up and periodical measurement of blood pressure of nephroticpatients to allow early recognition and management of hypertension