Glomerular diseases are important causes of morbidity and mortality among children in developing countries. Renal biopsy is the gold standard for determining histological diagnosis, prognosis, and ...therapy options. This study aimed to investigate the clinical, histopathological, and direct immunofluorescence findings of renal biopsy results in Nepalese children under 18 years old. In this retrospective cross-sectional study, the available data from children who had undergone kidney biopsy between 2016 and the end of 2020 were evaluated. Demographic data, indications of biopsy, histopathologic findings, and direct immunofluorescence findings were collected and presented. The mean age of the patients was 12.14 ± 4.07 years. Male/female ratio was 1:1. The majority of biopsy cases were aged between 11-15 years of age. The most common indication for biopsy in children was nephrotic syndrome (68.25%). Lupus nephritis with 28 cases (22.22%) had the highest frequency in overall renal biopsies. Minimal change disease (MCD) with 22 cases (17.46%) followed by Ig A nephropathy with 16 cases (12.69%) were the most frequent primary glomerulonephritis. Lupus nephritis showed full house positivity, and MCD showed full house negativity in all Direct immunofluorescence (DIF) parameters, whereas immunoglobulin A nephropathy showed 100% positivity in Ig A in DIF. Nephrotic syndrome was the most common indication for renal biopsy. The most common primary glomerulonephritis was MCD, while secondary glomerulonephritis was lupus nephritis. Clinical data, light microscopy, and direct DIF played an integral role in the overall final diagnosis.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
IgA nephropathy is one of the commonest forms of primary glomerulonephritis in the world, most commonly among Asian population. Though usually slowly progressive, it is one of the important causes ...of chronic renal failure. Abnormal IgA1 are formed which leads to formation of IgG antibodies which deposit in the mesangium. It presents with synpharyngitic hematuria and can have variable histopathological patterns. The Oxford classification was devised in order to categorize the histopathological patterns, correlate with clinical course and modify treatment accordingly. Different histopathological criteria are assessed in the classification, which include mesangial proliferation (M), endocapilary proliferation (E), segmental sclerosis (S), and interstitial fibrosis/tubular atrophy (T).The classification has become widely accepted around the world but still needs further validation studies and incorporation of newer parameters.
Background: Immunobullous disorders are a group of disorders involving the formation of a fluid filled cavity within or beneath the epidermis, due to the presence of autoantibodies against adhesion ...molecules in epidermis and dermis. Accurate diagnosis of these disorders requires clinicopathological correlation along with immunofluorescence study.
Aims and Objectives: This study was undertaken to describe the clinical features of immunobullous disorders and to analyse the utility of Direct immunofluorescence (DIF) in the diagnosis of these disorders.
Materials and Methods: A total of 42 Patients attending skin OPD between February 2014 and March 2017 who had a provisional diagnosis of immunobullous disease were enrolled in the study. Detailed clinical examination and DIF study were done in all cases.
Results: Out of 42 cases studied, 31 were diagnosed as pemphigus vulgaris (PV) and 11 as bullous pemphigoid (BP) that was confirmed by DIF. There were 20 (46.61%) male patients and 22 (52.38%) female patients in the age group of 18 to 81 years with a mean age of 52.64 years. A slightly female preponderance was observed. Mean age of presentation of PV patients is 50.83 years with age group range was between 18 to 77 years. Majority of patients presented at 4th and 5th decade of life. Age group range for BP was between 34 to 81years with mean age of presentation being 57.72 years. Majority of our patients presented at 5th decade or later. DIF was positive in all 42 cases (100%) of immunobullous disease. DIF in all 31 cases of PV showed 100% IgG deposition in intercellular substance (ICS) and 64.51% C3 deposition in ICS. BP showed 100% C3 deposition in all 11 cases, 63.63% IgG in seven of the eleven,18.18% IgA in two and 9% IgM, fibin in one each as a linear band at basement membrane zone (BMZ).
Conclusion: Both the clinical findings and the Imunofluorescence features are important in arriving at a definite diagnosis in immunobullous diseases. In all the cases DIF was absolutely essential tool to come to a final diagnosis.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Background: Tumours and tumour like lesions of the colon and rectum have overlapping clinical presentation and may be difficult to diagnose on clinical grounds alone. Histopathological examination ...although helpful to arrive at correct diagnosis, at times may be difficult and may require ancillary tests such as immunohistochemistry.
Materials and Methods: A prospective cross sectional study enrolled 125 cases of colorectal biopsies after histopathological confirmation of tumours and tumour like lesions. The specimens were received at the Department of Pathology, of TUTH, from December 1, 2015 to November 30, 2016.
Results: Out of 125 cases, 32 cases were benign tumours, 36 cases were malignant tumours and 57 cases were tumour like lesions. Most common age range was 51-60 years with mean age of 38 years. Males comprised 76.74% and females 23.26% with ratio of 3:1. Juvenile polyp was the commonest lesion. Among benign tumours, tubular adenoma was the most common (19.2%), adenocarcinoma was most common malignant tumour (25.6%) and juvenile polyp was the most common tumour like lesion (27.2%). Left side was most common side (66.67%) and rectum was the most common site (48.8%).
Conclusions: The commonest indications for colorectal biopsies were tumours and tumour like lesions. Juvenile polyp was the most common tumour like lesion, tubular adenoma was the most common benign tumour and adenocarcinoma was the most common malignant tumour. Histopathological examination is a gold standard for confirmation of all tumour and tumours like lesions of colon and rectum.
Background: Thalassemia and other structural hemoglobinopathies are the most common single gene disorders throughout the world with the highest frequency in the tropics, subtropics, Mediterranean ...basin and Southeast Asia. This study aims to provide a better assessment on the spectrum of hemoglobinopathies in our context.
Materials and Methods: This descriptive study was done at our tertiary care center, from November 2014 to October 2015. Hematological indices were derived from coulter counter, haemoglobin electrophoresis was carried out by cellulose acetate medium at alkaline PH. Sickling test, Hb H inclusions demonstration and Hb F estimation were performed. Parental screening was done wherever feasible.
Results: Out of 350 cases referred from various out-patient departments of TUTH and different peripheral hospitals for suspected hemoglobinopathies, 97 cases (27.71%) had hemoglobinopathies. The most predominant hemoglobinopathy was thalassemia (57.73%) followed by sickle cell disorders (28.87%) and Hb D/Hb E hemoglobinopathies (13.40%). Most disorders were observed in the age group 21-30 years with many of the cases seen clustered in the families and a slight male preponderance. Terai region (71.13%) had a very high number. A significant high frequency of thalassemia (33.93%) and sickle cell disorders (75%) were seen in Tharu community.
Conclusion: Hemoglobinopathies were seen widespread in Nepal. An extensive screening of the population is important to assess the prevalence of hemoglobinopathies, which will help in identification of carriers and take adequate therapeutic and preventive measures.
Background
Pericardial cysts are rare, with the most common etiology being congenital. Ventricular septal defect is the most common congenital heart disease in children. However, the combination of ...pericardial cyst, ventricular septal defect, and patent ductus arteriosus is extremely rare.
Case presentation
A one-year-old boy with ventricular septal defect and patent ductus arteriosus was planned for surgical correction. Intraoperatively, we made an additional diagnosis of a large pericardial cyst; and the cyst was excised along with ventricular septal defect closure and patent ductus arteriosus ligation.
Conclusions
Pericardial cysts can sometimes be missed with transthoracic echocardiography. Excision of the cyst can safely be done during concomitant cardiac surgery.
Background: Thrombocytopenia can be seen in any patients irrespective of age and the causes are diverse. This study was done to determine the frequency of various conditions associated with ...thrombocytopenia and to assess the number and morphology of megakaryocytes in various cases of thrombocytopenia. Megakaryocytic alterations in myelodysplastic versus non-myelodysplastic conditions were also evaluated in this study.
Materials and methods: This was a prospective study conducted on 132 cases of bone marrow aspirates of thrombocytopenia over a duration of one year from 14th April 2017 to 13th April 2018 in Tribhuvan University Teaching Hospital, Kathmandu. All the statistical evaluation was done by using SPSS version 24.
Results: In this study of 132 cases, megakaryocytic thrombocytopenia was the most common cause of thrombocytopenia for which bone marrow aspiration was done. The most common dysplastic change observed in megakaryocytes was micro megakaryocyte which was more commonly seen in MDS. Nondysplastic changes frequently observed were immature forms followed by bare nuclei and were more frequently seen in megakaryocytic thrombocytopenia followed by in myelodysplastic syndromes.
Conclusions: Many similar morphological changes were observed in megakaryocytes among different hematological diseases. So, the presence of dysplastic megakaryocyte should not prompt an interpretation of myelodysplastic syndromes and should always be correlated with patient’s clinical and hematological parameter.
Background. Severe complications of acute appendicitis (AA) hitherto well described are less common in clinical practice nowadays. When a septic child is encountered with a short history of abdominal ...symptoms and disproportionate signs of peritonitis further complicated by radiological findings causing a diagnostic conundrum, management becomes exceedingly demanding. Case Presentation. A 10-year-old previously healthy boy presented to the emergency room with generalized abdominal pain associated with fever and jaundice for a day. Blood workup revealed leucopenia, hyperbilirubinemia, hyponatremia, and elevated CRP. Initial radiological evaluation suggested hollow viscous perforation. He was diagnosed to have hollow viscous perforation peritonitis in severe sepsis. At laparotomy, generalized peritoneal contamination was found, the source of which could be traced down to the gangrenous perforated appendix. Conclusion. Complicated appendicitis, in children, can present with baffling findings. Timely identification of an ill child, adequate workup, prompt resuscitation, and source control are imperative for a successful outcome.
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DOBA, FZAB, GIS, IJS, IZUM, KILJ, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBMB, SIK, UILJ, UKNU, UL, UM, UPUK
Emergence of antibacterial resistance and production of Extended spectrum β-lactamases (ESBLs) are responsible for the frequently observed empirical therapy failures. Most countries have experienced ...rapid dissemination of ESBLs producing Enterobacteriaceae isolates, particularly E. coli and Klebsiella pneumoniae. ESBLs are clinically significant and when detected, indicate the need for the use of appropriate antibacterial agents. But antibacterial choice is often complicated by multi-resistance.
This study was carried from June to November 2014 to study the multidrug resistant (MDR) Enterobacteriaceae and ESBL producing E. coli among urine isolates in hospital setting. Isolates from urine samples were primarily screened for possible ESBL production followed by phenotypic confirmation. Antibiotic susceptibility testing (AST) was done by Kirby Bauer disk diffusion method following Clinical and Laboratory Standard Institute (CLSI) guidelines.
Out of 450 urine samples processed, 141 significant growths were obtained including 95 Enterobacteriaceae isolates with 67 E. coli. Among Enterobacteriaceae, 92 (96.84 %) were recorded as MDR and 18 (26.87 %) E. coli were confirmed as ESBLs producers.
Using the phenotypic confirmatory test forwarded by the CLSI, relatively significant E. coli isolates tested were ESBL producers. Also high numbers of MDR organisms were isolated among Enterobacteriaceae. Isolates showed significant resistance to the commonly prescribed drugs. These findings suggest for further study in this field including the consequences of colonization with MDR and ESBL-producing bacteria both in the community and in the hospital setting.
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