Dense Deposit Disease: A 29-Years Electron Microscopy Experience

Introduction: Dense Deposit Disease (DDD) is a devastating renal disease that leads to renal failure within 10 years of diagnosis in about half of affected patients. In this study, we evaluated the relative prevalence and pathological features of DDD diagnosed at our center over a 29 years period.Methods: We reviewed the clinical and pathological features of all cases of DDD diagnosed at the Electron Microscopy Unit of Ain Shams University (ASUSH) between January 1983 and December 2011.Results: From a total of 3283 renal biopsies, 33 (1%) were diagnosed with DDD (10 children and 23 adults). Nephrotic syndrome was the predominant clinical presentation of DDD (51.5%), and was commoner in children than adults (80% vs. 43%, p=0.03). Capillary wall thickening was seen in all cases (100%). Crescents were more commonly seen in children than adults (70% vs. 21.7%, p=0.008) while interstitial fibrosis was more commonly seen in adults (78.3% vs. 40%, p=0.03). The commonest histological pattern seen under light microscopy was membranoproliferative (27.3%), followed by crescentic (21.2%), membranous (21.2%), diffuse proliferative (18.2%), lobular (6.1%) and mesangial proliferative (6.1%) patterns. Immunohistochemistry was available for 25 cases and showed intense linear staining for C3 along capillary walls. Electron microscopic examination revealed glomerular basement membrane (GBM) thickening and intra-membranous and tubular basement membrane deposits in all cases (100%).Conclusion: DDD is a rare disease in Egypt, found in only 1% of renal biopsies. Pathological features of the disease differ between adults and children; children have predominantly glomerular damage whereas tubule-interstitial lesions are more often encountered in adults.Key words: Dense Deposit Disease; Electron Microscopy; Membranoproliferative Glomerulonephritis; Nephrotic Syndrom

Mitochondrial alterations in children with chronic liver disease

Background: Over recent years it has become apparent that the hepatocyte mitochondrion functions both as a cause and as a target of liver injury. Resultant dysfunction of mitochondria yields deficient oxidative phosphorylation, increased generation of reactive oxygen species, impairment of other metabolic pathways and activation of both necrotic and apoptotic pathways of cellular death.Methods: This study was conducted on 26 children and adolescents with chronic liver disease who presented to or were following up in the Pediatric Hepatology Clinic, Children’s Hospital, Ain-Shams University. They were divided into three groups according to the aetiology of liver disease (GI=patients with Wilson’s disease (WD), GII=patients with chronic hepatitis C, GIII=patients with chronic liver disease other thanWilson’s and chronic hepatitis C).Ultrasound-guided gun liver biopsies were performed, under local anaesthesia for all the 26 patients, using a modified 18-gauge truecut needle. Two liver biopsy cores were taken from each patient. One for light and electron microscopic examinations and the other was immediately immersed in liquid nitrogen to be frozen and used forstudying mitochondrial DNA deletions by PCR.Results: Liver steatosis was higher in the group of patients with Wilson’s disease and other liver disease. Electron microscopic examination of the mitochondria revealed significant mitochondrial pleomorphism in patients with Wilson’s disease and patients with chronic hepatitis C infection. Enlarged mitochondria were found to bemore prevalent among patients with chronic  hepatitis infection.Three of our patients (11.53%) had mitochondrial DNA deletions. We developed scoring system for mitochondrial affection in our patients, 7 patients (32%) were considered to have mild mitochondrial affection,9 patients (41%) had moderate mitochondrial affection, while 6 patients (27%) had severe mitochondrial affection. Four of the studied patients had no mitochondrial affection.Conclusion: Mitochondria affection is common in chronic liver disease. This mitochondrial affection might be responsible for some of the chronic liver disease manifestation such as easy fatiguability and steatosis

Characteristics and outcomes of COVID-19 patients admitted to hospital with and without respiratory symptoms

Background: COVID-19 is primarily known as a respiratory illness; however, many patients present to hospital without respiratory symptoms. The association between non-respiratory presentations of COVID-19 and outcomes remains unclear. We investigated risk factors and clinical outcomes in patients with no respiratory symptoms (NRS) and respiratory symptoms (RS) at hospital admission. Methods: This study describes clinical features, physiological parameters, and outcomes of hospitalised COVID-19 patients, stratified by the presence or absence of respiratory symptoms at hospital admission. RS patients had one or more of: cough, shortness of breath, sore throat, runny nose or wheezing; while NRS patients did not. Results: Of 178,640 patients in the study, 86.4 % presented with RS, while 13.6 % had NRS. NRS patients were older (median age: NRS: 74 vs RS: 65) and less likely to be admitted to the ICU (NRS: 36.7 % vs RS: 37.5 %). NRS patients had a higher crude in-hospital case-fatality ratio (NRS 41.1 % vs. RS 32.0 %), but a lower risk of death after adjusting for confounders (HR 0.88 [0.83-0.93]). Conclusion: Approximately one in seven COVID-19 patients presented at hospital admission without respiratory symptoms. These patients were older, had lower ICU admission rates, and had a lower risk of in-hospital mortality after adjusting for confounders