Eye of the Tiger Sign in Pantothenate Kinase-Associated Neurodegeneration.

Pantothenate kinase-associated neurodegeneration (PKAN) is a rare disorder associated with brain iron accumulation caused by a recessive mutation in pantothenate kinase 2 gene (PANK2). We present a case of an 11 year-old boy presenting extrapyramidal signs and developmental regression. T2-weighted images showed the classic eye of the tiger sign seen in pantothenate kinase-associated neurodegeneration.

[Bilateral stellate neuroretinitis as presenting sign of pheochromocytoma]. / Neurorétinite stellaire bilatérale révélant un phéochromocytome.

We report the case of a 21-year-old male patient admitted emergently with progressive bilateral severe visual loss for 1 month. Posterior segment examination revealed bilateral stellate neuroretinitis. Infectious serologies were negative, and brain CT was normal. Physical examination was remarkable for malignant hypertension of 220/150 mmHg. Diagnostic work-up revealed a pheochromocytoma documented by histopathological exam upon adrenalectomy. The disc edema and macular exudates resolved once the hypertension was controlled.

Induction of defense responses in common bean plants by arbuscular mycorrhizal fungi.

Interaction between arbuscular mycorrhizal fungi as a bio-agent and Rhizoctonia root rot disease of common bean plant was investigated in this study under natural conditions in pot experiment. A mixture of Egyptian formulated AM (Multi-VAM) in suspension form (1 × 10(6) unit L(-1) in concentration) was used at dilution of 5 ml L(-1) water. The results demonstrated that colonization of bean plants with AM fungi significantly increased growth parameters, yield parameters and mineral nutrient concentrations and reduced the negative effects on these parameters as well as both disease severity and disease incidence. Different physical and biochemical mechanisms have been shown to play a role in enhancement of plant resistance against Rhizoctonia solani, namely, improved plant nutrition, improved plant growth, increase in cell wall thickening, cytoplasmic granulation, and accumulation of some antimicrobial substances (phenolic compounds and defense related enzymes).

The role of radiotherapy in the treatment of pterygium: a review of the literature including more than 6000 treated lesions.

Pterygium is a benign conjunctival neoformation usually treated by surgical excision, but recurrences may affect 30% to 89% of cases, so that adjunctive therapies like conjunctival autografting, antimitotic drugs and beta-irradiation (ß-irradiation) are often used to improve the rate of local control. Our essay has reviewed relevant studies addressing the role of postoperative irradiation in the treatment of pterygium in the last 30 years through an Internet-based search and hand search in libraries. Sixteen studies on ß-irradiation and one on soft X-ray irradiation were accessible. They covered more than 6000 lesions treated by surgical excision and postoperative ß-irradiation using strontium-90 ((90)Sr) applicators at doses varying from 10 to 60 Gy/1-6 fractions/1-6 weeks starting within 3 days postoperatively. The rates of local recurrence were in general lower than 15% and major complications such as scleral thinning, ulceration, infections, or radiation-induced cataract were rarely encountered. Early postoperative ß-irradiation at a dose of 30 Gy/three fractions/2-3 weeks starting within 24h from surgical excision is an effective and safe procedure with local control rates comparable to chemotherapeutic agents and conjunctival autografting and superior to simple excision alone.

Tenascin and strictures in inflammatory bowel disease: an immunohistochemical study.

Tenascin is an extracellular matrix protein involved in morphogenesis of muscle tissue and in wound healing. In the present study we examined its distribution in tissue from patients with inflammatory bowel disease. Intestinal biopsies from 10 normal controls, 15 patients with Crohn's disease, and 6 with ulcerative colitis were studied. Samples were obtained both from uninvolved and involved areas. Mucosal tenascin is increased in ulcerative colitis and Crohn's disease, especially in areas of ulceration. In Crohn's disease, tenascin is also strongly expressed in the submucosa and in smooth muscle cells of the muscularis mucosae and propria, especially in areas of stricture. We conclude that tenascin is involved in stricture formation in Crohn's disease and that it is a marker of phenotypic change in smooth muscle cells.

The association of Helicobacter pylori infection with the development of gastroesophageal reflux disease (GERD).

A large number of studies were performed that support the association of Helicobacter pylori infection and the development of (GERD), although to prove that this association is a causation is still a matter of dispute. The objectives of this study was to prove this causal association in order to be able to plan a program for intervention. The work was conducted on 30 patients suffering from symptoms suggestive of gastro-esophogeal reflux disease diagnosed by upper endoscopic examination and 20 matched controls were chosen complaining from dyspeptic symptoms but with completely normal by upper endoscopic examination. The patients and controls were selected from Mataria Teaching Hospital and Ain Shams Specialized Hospital in Cairo and subjected to complete history taking, clinical examination, endoscopic examination and biopsy from the lower end of esophagus for examination of the presence of Helicobacter Pylon. No causal association between infection with Helicobacter pylon and the development of (GERD) could be demonstrated. It is highly recommended to further investigate this association in patients with grade IV esophagitis (Barrett's esophagus) only.

Renographic indices for evaluation of changes in graft function.

Radionuclide renal diagnostic studies play an important role in assessing renal allograft function, especially in the early post-transplant period. In the past two decades various quantitative parameters have been derived from the radionuclide renogram to evaluate changes in perfusion and/or function of the kidney allograft. In this review article we discuss the quantitative parameters that have been used to assess graft condition, with emphasis on the early postoperative period. These quantitative methods are divided into parameters used for assessing renal graft perfusion and parameters used for evaluating parenchymal function. The blood flow in renal transplants can be quantified (a) by measuring the rate of activity appearance in the kidney graft, (b) by calculating the ratio of the integral activity under the transplanted kidney and arterial curves and (c) by calculating the renal vascular transit time. In this article we review a number of parenchymal uptake and excretion indices, such as the accumulation index, the graft uptake capacity at 2 and 10 min, the excretion index and the elimination index. The literature on these parameters shows that they have some practical disadvantages. In addition, values suffer from significant overlap when various graft pathologies coexist. A retrospective study was designed in our institution to evaluate the clinical usefulness of some of the frequently used previously published methods in which the graft function is quantitatively assessed in the early post-transplant period. The quantitative parameters studied which were reasonably reproducible in our hands included: global perfusion index (GPI), cortical perfusion index (CPI), vascular transit time, and the parenchymal parameters uptake capacity at 2 min (UC2) and elimination index (K3/20). The patient population in this study consisted of 43 patients with 157 technetium-99m mercaptylacetyltriglycine renograms. The perfusion indices GPI and CPI did not allow differentiation of the acute tubular necrosis (ATN) group from the acute rejection (AR) group; however, they were of value in monitoring the improvement in the condition of the graft dysfunction in both the AR and ATN groups. As for the parenchymal parameters, both UC2 and K3/20 were able to differentiate stable graft function (SGF) versus AR and ATN groups but were unable to separate AR from ATN dysfunction. The ability of these parenchymal parameters to detect improvement in the graft function was poor and statistically non-significant. From the literature data and our own findings it is concluded that radionuclide scintigraphy of renal transplants has assumed an important role, especially if performed serially, in monitoring graft function in the post-transplant period. Many quantitative parameters have been derived from the radionuclide renogram to evaluate changes in perfusion and/or function of the kidney allograft. It appears that these quantitative numerical values are unable to differentiate unequivocally between grafts with ATN and AR cases. The real value of these parameters lies in the follow-up of the dysfunction processes, which helps the clinician to determine the appropriate therapeutic regimen.

Vascular alterations of the colonic mucosa in schistosomiasis and portal colopathy.

BACKGROUND/AIM: Portal colopathy, the occurrence of vascular-ectasia like lesions has been observed in patients with portal hypertension of variable etiology. Schistosomiasis is a major cause of liver damage and portal hypertension. In colonic schistosomiasis, vascular alterations are commonly observed. It is therefore possible that schistosomiasis may induce portal colopathy in addition to inflammatory changes directly induced by oviposition. MATERIALS AND METHODS: In order to examine this possibility, we reviewed the endoscopic data obtained in 100 consecutive patients with established bilharziosis. In addition, endoscopic biopsies from all patients were examined for the presence of inflammation, parasite eggs, granulomas and mucosal vascular congestion. The latter was assessed using immunohistochemical staining for Ulex Europaeus. RESULTS: Endoscopic abnormalities were observed in 66/100 patients. The main lesions were abnormalities in vascularisation of the mucosa, especially hyperemia, defined as the presence of numerous, prominent and irregular vessels (62%) and telangiectasia (4%). The mucosal biopsies revealed prominent vascularisation in 60% of the cases. Positivity for ulex staining was significantly correlated with the finding of hyperemia during endoscopy and with the presence of ova. No good correlation was found with the clinical presentation. The lesions were not well correlated with the presence of an increased cellular infiltrate in mucosal biopsies. CONCLUSIONS: This observation suggests that portal colopathy may explain some of the endoscopic lesions observed in the colon of patients with Schistosomiasis.

Detection of Schistosoma mansoni antigen in the liver and antibodies in serum of patients with hepatic schistosomiasis.

Liver biopsies were taken from 28 patients with chronic hepatitic schistosomiasis for histopathological study and S. mansoni antigen detection by indirect immunofluorescence test. Circulating antibodies were estimated by indirect haemagglutination test. Thirteen cases (46.4%) revealed diffuse S. mansoni antigen in the liver blood sinusoids and in the formed granulomata. Circulating antibodies were detected in 21 cases, 11 of them showed antigen in the liver. Five cases only revealed definite granulomatous reactions around bilharzial ova, while the remaining 23 cases showed periportal fibrosis. So, for proper diagnosis of chronic hepatic schistosomiasis, it is recommended to examine the liver for S. mansoni antigen together with circulating antibodies detection.

Controlled trial with interferon alfa 2-b in chronic hepatitis C patients.

Thirty-eight chronic hepatitis C (CHC) Egyptian patients with persistently elevated serum alanine aminotransferase (ALT) for 6 months were randomly allocated into 2 groups: Group I (19 patients) received 3 million units (MU) of interferon alpha - 2b (Intron-A) subcutaneously thrice weekly for 6 months. In group I, complete response (normalization of ALT by the end of treatment) was achieved in 8 patients (42.1%), partial response (decrease of ALT by at least 50% of the pretreatment values) in 7 patients (36.8%) and no response in 4 (21.1%). Sustained response for 6 months after the end of therapy was attained in 4 of the 8 (50%) complete responders. Thus attaining an overall sustained response in 4 of the 19 patients (21.1%). In group II, spontaneous normalization of ALT was established in 1 patient (5.3%). Repeat liver biopsies in 16 patients of the interferon group, revealed moderate improvement in the degree of lobular inflammation, hepatocyte necrosis and portal inflammation. We conclude from this study that treatment of CHC with 3 MU of IFN-alfa 2b thrice weekly for 6 months is associated with a low response rate (21.1%). To improve the results, escalation of the IFN dose and/or prolongation of the treatment period should be considered.

Impact of nutritional supplementation on treatment delay and morbidity in patients with head and neck tumors treated with irradiation.

Twenty-three patients with head and neck cancer were randomly allocated, regardless of pretreatment nutrition status, to receive either radiotherapy alone (12 patients) or radiotherapy plus nutritional oral supplementation (11 patients) with high-protein nutritional powder of balanced nutrient content (Ensure). Nutrition status was assessed subjectively and objectively for all patients before and at weekly intervals during the course of irradiation. All patients who received nutritional supplementation experienced an increase in body weight and in triceps skin-fold thickness, whereas 58% of the other group had weight loss (p = 0.001). Irradiation had to be suspended in 5 of the 12 patients who received no nutritional support because of severe mucosal reaction and/or poor performance status. All patients who received nutritional supplementation during radiotherapy had their course of irradiation without interruption.

Radionuclide study of the blood ocular barrier.

The purpose of this study was to investigate the permeability and clinical significance of the blood-ocular barrier. A new technique using systemic, subtenon, and retrobulbar injections was applied to the study of intraocular penetration of labelled steroid compound. The study was carried out in normal rabbits and in animals with artificially induced intraventional inflammation. It is concluded that there is no blood-vitreous barrier, a vague concept in any case, in the anterior region of the vitreous where free diffusion was observed between the anterior and posterior segments of the eye. High concentration of the labelled steroids was found in the cornea with the peak of activity usually at 30 minutes. However, traces of activity were detectable for up to 4 hours. This indicates the need for a depot long term respiratory form of corticosteroid that would deliver a high concentration of the medication. Local subtenon's and retrobulbar injections resulted in a relatively higher ocular I-125 Cortisol concentration than obtained by systemic route. It is concluded therefore that a high concentration of steroids applied locally will give better and less deletrious effects than systemic administration. Subtenon injection resulted in higher activity in various ocular tissues especially aqueous and vitreous. However, the I-125 Cortisol was rapidly cleared regardless of the route of administration.

Clinico-epidemiological study of Kaposi's sarcoma in Egypt.

The clinico-epidemiological aspects of Kaposi's sarcoma are reviewed. A study of 27 patients seen in Egypt during the years 1962--78 is reported. The treatment modalities are discussed with special reference to radiotherapeutic management.

Immune deficiency in Hodgkin and non-Hodgkin lymphoma.

Forty-three previously untreated Egyptian patients with Hodgkin's disease and thirty-five patients with non-Hodgkin lymphoma were studied with several readily available tests of immune function, number of peripheral blood lymphocytes, delayed hypersensitivity to two recall antigens, in vitro blastoid transformation by PHA, the capacity of E-rosette formation and surface marker criteria. The results were correlated to the histology, stage of disease and to the presence of general symptoms and signs.

Value of serum copper measurement in acute leukaemia of childhood.

Serum copper level (SCL) determination was carried out on sixteen normal children and on 16 with acute leukaemia. A significant increase in SCL was observed in cases of leukaemia than in normal controls. Drop of SCL occurs in cases who respond to quadruple chemotherapy, while those who failed to respond showed persistantly high serum copper level.