Multidisciplinary approach for evaluation of neurocutaneous disorders in children in Sohag University Hospital, Upper Egypt

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

Effect of some plants as functional food supplement on kidney function and structure in experimentally hyperuricemic rats

Hyperuricemia is associated with renal insufficiency and commonly used as a useful early biomarker for chronic kidney diseases. Celery leaves contain a valuable volatile oil and an organic substance called apiol. Celery leaves, chicory and barley are known to have beneficed effect for humans in health and disease. This work was carried out to investigate the effect of dietary fiber supplementation with celery, chicory and barley, alone and in combination, on body weight, food efficiency radio, kidney function and renal histology in experimentally hyperuricemic rats. The experiment was performed using 4 groups of Sprague Dawley rats fed on basal diet supplemented with potassium oxonate [uricase inhibitor] at 2% for 6 weeks to induce experimental hyperuricemia and one group was fed on basal diet only and used as a negative control group. Four experimental diets were prepared by adding the dry powder of celery seeds, chicory leaves or barley grains at 10% or mixture of three plants at 15% [5% from each] to basal diet. The feeding period was 4 weeks. Biochemical analysis of blood urea nitrogen, uric acid and creatinine concentrations in the serum as well as histopathological examination of kidney were performed. Results showed that feeding of celery, chicory and barley mixture at 15% to hyperuricemic rats for 4 weeks increased body weight and improved feed efficiency. It decreased the elevated serum urea nitrogen, uric acid and creatinine concentrations. It also produced an excellent effect on renal histology as it ameliorated the renal damage caused by elevated serum uric acid. The study suggests that dietary intake of plant mixture of celery, chicory and barley at 15% concentration for 4 weeks may be beneficial to patients suffering from hyperuricemia and kidney disease because it improves the function and structure of kidney in hyperuricemic rats

Effect of parsley on both healthy rats and those suffering from acute renal failure

The Present work was conducted to study the effect of parsley on both health rats and those suffering from acute renal failure [ARF] on the nutritional value, the relative ratio of organs to body weight, blood lipid parameters, kidney and liver functions. Thirty male albino rats [Sprague - Dawaley strain] weighting [200 +/- 5 g] divided into three main groups. The first main group [5 rats] fed on basal diet [control negative group]. The second main group [10 rats] divided to two subgroups and fed on basal diets containing 2% and 5% dried parsley. The third main group [15 rats] injected subcutaneous with glycerol [50%, W/v in saline solution] 10 ml/ kg body weight to induce acute renal failure [ARF] and divided into three subgroups, the first fed on basal diet as a [control positive], while second and third subgroups fed on basal diet containing 2% and 5% dried parsley, respectively. The result revealed that, feeding rats, which suffering from ARF on basal diet containing dried parsley [2% and 5%] resulted in improvement on the nutritional value as well as healthy groups, organs weight/body weight relative ratio, serum cholesterol, triglycerides, low density lipoprotein cholesterol [LDL-c], high density lipoprotein cholesterol [HDL-c], very low density lipoprotein cholesterol [VLDL-c], uric acid, urea nitrogen, aspartate amino transferase [AST] and alanine amino transferase [ALT] than the control positive group. It was concluded that parsley at high concentration 5% realized the best effect on lipid profile, kidney and liver functions

Colonic bilharzioma; surgical intervention versus conservative treatment

Bilharzioma implies a localized mass of fibrous and inflammatory tissue, which usually contains many eggs, frequently involving serosa and mesentery. Bilharzioma is probably caused by reaction to numerous eggs produced by one or more pair of worms in a single site. The aim of this study is to compare between surgical intervention and conservative treatment in a group of patients had colonic bilharzioma presented with abdominal masses and intestinal obstruction. Ten patients presented with abdominal masses and intestinal obstruction. Complete blood count, urine analysis and stool analysis done to all the patients of the study. Abdominal ultrasonogiaphy done in all the patients of the study. Abdominal computed tomography done in 6 patients. Barium enema done in 5 patients. All the patients of the study had been subjected to laparotomy. There were 7 males and 3 females, their ages ranged from 8 to 42 years [mean 14.2 years]. All the patients had abdominal pain [100%], 6 patients presented with abdominal mass [60%], and 4 patients presented with intestinal obstruction [40%]. There was microcytic hypachromic anemia in 9 patients [90%] and leukocytosis with eosinophilia in 7 patients [70%]. Abdominal US revealed presence of lymphoma versus teratoma in 6 patients [60%] and signs of intestinal obstruction in 4 patients [40%]. Barium enema revealed presence of multiple polyps throughout rectosigmoid colon, with loss of haustrations and spasm of descending colon in 3 cases. Colonic wall thickening with narrowing and rigidity of the ascending colon in 2 cases. Abdominal CT revealed presence of colonic wall masses suggestive of lymphoma in 6 cases. In laparotomy, there were 4 rectosigmoid bilharzioma, bilharzioma of the transverse colon down to the upper rectum in 3 patients and ascending colonic bilharzioma in 3 cases. Histopathology confirmed presence of bilharzial granuloma in all surgical specimens. Always consider bilharzioma in differential diagnosis of abdominal masses and intestinal obstruction, especially in countries where it is endemic.Biopsy is a must as clinical examination laboratory and radiological methods are not diagnostic

Surgical management of complicated hepatic hydatid cysts

Hydatid disease is a parasitic infestation caused by echinococcus granulosus characterized by cystic lesions in the liver, lungs, and rarely in other parts of the body. Yemen is highly endemic area for hydatid disease, especially in the northern areas with sheep-raising. Surgery is the cornerstone of management of hydatid cyst liver and aims to remove the cyst or its remnants and obliterate the residual cavity. The aim of this study is to identify the optimum and safest way for treating patients having complicated hydatid cyst liver with the least possible complications. This study includes characteristics on presentation, investigations, operative techniques, postoperative morbidity and mortality in a group of patients with complicated hydatid cyst liver. Twenty-two patients with complicated hydatid cyst liver were included in this study, ages ranged from four years to sixty-five years. These patients were managed over a period of 12 months, investigated by plain chest X-ray, abdominal ultrasonography and abdominal computerized tomography. Operative procedure was the rule, after correction of the general condition of the patients, where complete cystectomy was done in two patients, segmentectony was done in one patient, partial pericystectomy+capitonnage was done in three patients, partial pericystectomy+omentoplasty in nine patients and partial pericystectomy+external drainage in seven patients. Fourteen patients had a cyst on the right lobe, 6 patients ,had a cyst on the left lobe, and 2 patients had a cyst on both lobes. As regards surgical procedures, the patients were divided into 5 groups according to the type of operation; Group one consists of 2 patients with peripherally located small cysts. They underwent cystectomy. Group two consists of one patient underwent segmentectony, Group three consists of three patients underwent partial pericystectomy+capitonnage, group four consists of nine patients underwent partial pericystectomy+omentoplasty, Group five consists of seven patients underwent partial pericystectomy+external drainage, There is one postoperative death from postoperative hepatic failure. The principle of complete excision of the cyst, partial pericystectomy+capitonnage or omentoplasty or external drainage is applied in this study where complete cystectomy is the technique of choice in selected patients, as it is a associated with low morbidity and short hospital stay

Insulin-like growth factor [IGF] -1, IGF-II, IGF-binding protein -1 [IGFBP-1] and IGFBP-3 in children with insulin dependent diabetes mellitus

The existing literature on serum insulin like growth factors [IGFs] and their binding proteins [IGFBPs] levels in insulin dependent diabetes mellitus [IDDM] is conflicting. In the present study, 44 children and adolescents with IDDM [22 newly diagnosed and 22 old known cases] aged from 5 to 14 years, 18 girls and 26 boys, together with 18 apparently healthy control children of matchable age and sex were subjected to estimation of serum levels of IGF-I, IGF II, IGFBP-1, IGFBP-3 and fasting serum glucose. Glycemic control was assessed by glycosylated hemoglobin [HbA[1C]]. Diabetic children showed significantly lower serum levels of IGF-I, IGF-II and IGFBP-3 [p<0.01, p<0.01 and p<0.001 respectively] and significantly higher levels of IGFBP-1 [p<0.05] than controls. This was true for the diabetic cases in general, for newly diagnosed cases and for those with poor metabolic control. Old diabetic cases and patients with average glycemic control differed from the control children only regarding IGF-II [p<0.001 for both] and regarding IGFBP-3 [p<0.001] and p<0.05 respectively]. While prepubertal cases showed significantly lower IGF-I than controls [p<0.05], pubertal cases lacked this difference. IGF-I levels were negatively correlated with each of HbA[1C] [r=-0.52, p<0.001] and IGFBP-1 [r=-0.45, p<0.002], and were positively correlated with each of IGFBP 3 [r=0.31, p<0.05] and body mass index [BMI] [r=0.43, p<0.004]. IGFBP-1 levels were positively correlated with fasting serum glucose [r=0.46, p<0.002]. In conclusion, alterations in the IGF/IGFBP system are observed in children with IDDM in the present study. Improvement of glycemic control may help to decrease this alteration. The impact of these alterations on the health of diabetic children is still unsettled

Role of pulmonary arteriography in pulmonary embolism

The majority of episodes of deep venous thrombosis are silent, and the manifestations of pulmonary embolism are often non specific, so that the clinical suspicion and accurate diagnosis are mandatory. Ventillation/Perfusion [V/Q] scintigraphy is a non invasive test of reasonably high diagnostic accuracy [Secker-Walker, 1983]. Isotopic scan results should be correlated with a recent chest x-ray, as this increase the efficacy of the test [Secker-Walker, 1983]. On a plain chest film several signs are helpful but there is no pathognomonic radiologic sign that can be relied upon with complete confidence. In developing counteries, there is the problem of regular supply of good ventillation agents. Furthurmore, the [V/Q] scans are sometimes equivocal. Pulmonary arteriography may offer the only possibility of making an absolute diagnosis of pulmonary embolism. In this study pulmonary arteriography was performed for 29 patients, 21 males and 8 females their ages ranging between 30 - 70 years. The technique being relatively invasive, in this article we state our own experience regarding the necessity, diagnostic yield, hazards and safety of the procedure