ABSTRACT
Neurocutaneous syndromes [NCS] are a broad term for a group of neurologic disorders that involve the nervous system and the skin. The most common examples are neu-rofibromatosis type 1 [NF-1] and type 2 [NF-2], tuberous sclerosis [TS], Sturge-Weber syndrome [SWS], ataxia telangiectasia [AT], and Von Hippel Lindau disease [VHL]. These disorders are characterized clinically by neurological manifestations such as convulsions, mental retardation and learning disabilities in addition to cutaneous manifestations, and lastly tubers [benign growths found in different organs of the body]. This study aimed to identify clinical, imaging, and neurophysiological profiles of neurocutaneous disorders. Children presented to the Pediatric neurology and Dermatology clinics, Sohag University Hospital who fulfilled the criteria for diagnosis of specific neurocutaneous syndromes were eligible for this study. All studied patients were subjected to thorough clinical history, full clinical examination, developmental assessment, and dermatological examination. Computed tomography of the brain [CT] and electroencephalography [EEG], ophthalmic, and phoniatric evaluation were also done for all children. Echocardiography was done for only twenty children. During the period of the study we diagnosed 27 cases with neurocutaneous disorders, tuberous sclerosis represented the majority of cases as it was detected in 12 cases [44.45%]. The main complaint was convulsions in 19 cases [70.37%], whereas skin pigmentation was detected in 18 cases [66.66%]. Developmental assessment showed that global developmental delay was found in 20 cases [74%]. CT of the brain showed that 15 cases [55.55%] had intracranial calcifications and abnormal EEG findings were detected in 23 cases [85.2%]. 85% of the studied children had various degrees of mental retardation. Echocardiography showed that three cases [15%] had ventricular wall tumor mostly rhabdomyoma Neurocutaneous disorders had multiple clinical presentations and required a team work approach including various specialties in their evaluation and management
ABSTRACT
To study the epidemiological, clinical and histopathological parameters of childhood idiopathic nephrotic syndrome in Sohag. This is a prospective cohort study conducted at Pediatric Nephrology Unit, Sohag University Hospital from Sept.2004 to March 2006. All patients with newly diagnosed nephrotic syndrome were eligible to this study. In addition to full history and clinical examination all patients were subjected to urine analysis, urinary albumin creatinine ratio, serum albumin, serum cholesterol and triglycerides, kidney function tests and complement [C3]. All patients received the conventional treatment of nephrotic syndrome [oral prednisolon 2 mg/kg/day in 3 divided doses for one month]. If the patient is respond the total daily dose of prednisolon was given as a single dose with breakfast every other day for 6 months]. If the patient do not show any response despite one month of daily treatment we did a renal biopsy. Patients who responded but have frequent relapses were also subjected to renal biopsy. A total of 134 children [84 males: 50 females] with nephrotic syndrome were enrolled into this study. Their mean age was 8.4 years +/- 1.8. Male to female ratio was 1.7:1. 110 patients [82.1%] showed initial response to steroid [steroid sensitive]. On follow up of these patients 28 patients [25.5%] had no relapse, 34 patients [30.9%] had infrequent relapses, 39 patients [35.5%] had frequent relapses, and 9 patients [8.2%] became late steroid non responders. 24 patients [17.9%] show resistance to steroid from the start [steroid resistant nephrotic syndrome]. Steroid dependency noted in 57 patients [51.8%] of all categories. 47 patients have been subjected to renal biopsy: 25 patients [53.2%] have Focal segmental glomerulosclerosis [FSGS], 11 patients [23.4%] have minimal change nephrotic syndrome [MCNS], 9 patients [19.1%] have mesangioproliferative [MPGN], and 2 patients [4.3%] have membranous glomerulonephritis [MGN]. Nephrotic syndrome is common in our locality. Clinical aspects and rate of response to treatment of our patients are similar to published studies. However our patients are different in histopathological aspects. Renal biopsy is an important, safe, and an accurate method for the diagnosis of selected cases of nephrotic syndrome
Subject(s)
Humans , Male , Female , Child , Signs and Symptoms , Kidney Function Tests , Kidney/pathology , Biopsy , HistologyABSTRACT
Surgical treatment is the gold standard method for treatment of infantile pyloric stenosis. Surgery, however, is not without risks. Medical treatment with atropine was introduced long time ago and seems to be an attractive alternative to surgery. To treat infantile pyloric stenosis medically or surgically is still a controversial area. To test whether medical treatment is as effective as surgery in treatment of infantile pyloric stenosis and to determine which infant is suitable for either modality. 28 infants with infantile pyloric stenosis were included in this study. In all infants the diagnosis was confirmed by an abdominal ultrasound and gastrographin study. Medical treatment consisted of intravenous atropine 0.01 mg/kg /dose every 4 hours followed by oral atropine 0.02 mg/kg/dose every 6 hours. Surgical treatment consisted of standard Ramstdt's pyloromyotomy under general anesthesia. 14 infants were treated medically and 14 infants were treated surgically. Medical treatment was successful in 8 infants [66%] whereas surgical treatment was successful in all cases [p <0.002]. Mean hospital stay was 10 +/- 2 days in medically treated infants and it was 3 +/- 1 days in surgically treated infants [P<0.0001]. In our Hospital, surgical treatment is superior to medical treatment in cases of infantile pyloric stenosis. However the encouraging success rate of medical treatment may justify trying it in infants waiting for surgery or in infants in whom surgery is contraindicated