scholarly journals IgA nephropathy and atypical anti GBM disease: A rare dual pathology in a pediatric RPGN

2021 ◽  
Author(s):  
Varun Bajaj ◽  
Shilpi Thakur ◽  
Adarsh Barwad ◽  
Aditi Sinha ◽  
Arvind Bagga ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Rapidly Progressive Glomerulonephritis ◽  
Age Group ◽  
Case Presentation ◽  
Plasma Exchange Therapy ◽  
Dual Pathology ◽  
Pediatric Age Group ◽  
Disease Case ◽  
Iga Antibodies

Introduction: Anti-GBM nephritis in the pediatric age group is exceedingly rare with concurrent additional pathologies being even rarer. Tissue diagnosis requires a combination of crescentic histomorphology, immunofluorescence showing “Paint brush stroke” pattern of linear IgG or rarely IgA and serum anti-GBM antibodies subject to the disease course and treatment. The authors describe one such case with a dual pathology involving IgA nephropathy and atypical anti-GBM disease. Case presentation: A thirteen-year-old girl presenting with features of rapidly progressive glomerulonephritis underwent a renal biopsy showing a mesangioproliferative histology with crescents and an immunofluorescence pattern indicating a dual pathology of IgA Nephropathy and Anti GBM Nephritis. Additional ancillary testing including staining for IgG subclasses and galactose deficient IgA (KM55) helped to confirm the diagnosis. She responded to steroid pulses and plasma exchange therapy, was off dialysis after 8 weeks with serum creatinine of 1.5 mg/dl however remains proteinuric at last follow up. Conclusion: Concurrent Anti-GBM nephritis and IgA nephropathy is a rare occurrence and possibly arises from a complex interaction between the anti-GBM antibodies and the basement membrane unmasking the antigens for IgA antibodies. Additional newer techniques like immunofluorescence for KM 55 are helpful in establishing the dual pathology.

scholarly journals PURE YOLK CELL TESTICULAR TUMOR;

2017 ◽  
Vol 24 (04) ◽  
pp. 637-638
Author(s):  
Muhammad Sheraz Javed ◽  
Muhammad Irfan Munir ◽  
Muhammad Saad Siddique
Keyword(s):  
Age Groups ◽  
Malignant Lesion ◽  
Germ Cell Tumors ◽  
Testicular Tumor ◽  
Cell Tumor ◽  
Age Group ◽  
Case Presentation ◽  
Yolk Cell ◽  
Pediatric Age Group

Background: Testicular tumor primarily originate from germ cells and are foundin all age groups. Among germ cell tumors one is pure yolk cell tumor which is tumor of infantand pediatric age group and is extremely rare in adulthood. Case Presentation: Current titledcase report is about a 23 year old male who presented with painless enlargement of righttestis. Examination revealed as hard lump involving right testis and clinically epididymis spared.Hormonal assessment consistent with malignant lesion of testis. Right inguinal approachedorchidectomy done and histopathology revealed it as pure yolk sac tumor of testis. Conclusion:Pure yolk cell tumor in adulthood is a very rare tumor and once diagnosed, need follow up inpost-operative circumstances.

Ewing's Sarcoma: An Approach to Radiological Diagnosis

1979 ◽  
Vol 65 (3) ◽  
pp. 389-399 ◽  
Author(s):  
Fabrizio Lombardi ◽  
Marco Gasparini ◽  
Cristina Gianni ◽  
Raffaele Petrillo ◽  
John David Tesoro-Tess ◽  
...  
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Primary Site ◽  
Long Bones ◽  
Age Group ◽  
Pediatric Age Group ◽  
Localized Disease ◽  
The Mean ◽  
Small Bones

All the pertinent radiographs of 83 patients with histologically proven Ewing's sarcoma were reviewed. Forty-nine patients were in the pediatric age group, and 34 were adults. The mean age, the symptoms and time from symptoms to diagnosis were evaluated in the 2 groups. The site of primary involvement was in 54 % the long bones, 35 % the flat bones, 8 % the small bones and 3 % extraosseous. For the primary site we considered the diagnostic results of the standard radiographic investigations and in some cases the usefulness of angiography, xeroradiography and telethermography. At presentation we also evaluated the possible diffusion of the disease with standard radiographic surveys (chest and skeletal, including limbs) and with foot lymphography in selected cases. In this way, 57 patients (69 %) were considered to have localized disease. In this group, we also considered the value of the periodic radiographic follow-up, which enabled us to disclose the appearance of metastases (chest 64 %, bone 54 %, lymph nodes 11 %) in 28 cases (49 %). Finally, we made a comparison of the different radiologic and epidemiologic findings between children and adults.

scholarly journals Methotrexate Treatment in Children with Febrile Ulceronecrotic Mucha-Habermann Disease: Case Report and Literature Review

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Isil Bulur ◽  
Hilal Kaya Erdoğan ◽  
Zeynep Nurhan Saracoglu ◽  
Deniz Arık
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Relevant Literature ◽  
High Fever ◽  
Age Group ◽  
Pediatric Age ◽  
Pityriasis Lichenoides ◽  
Pediatric Age Group ◽  
Disease Case ◽  
Methotrexate Treatment

Febrile Ulceronecrotic Mucha-Habermann disease is a rare and potentially fatal variant of pityriasis lichenoides et varioliformis acuta and is characterized by high fever, constitutional symptoms, and acute oncet of ulceronecrotic lesions. We present an 11-year-old male with Febrile Ulceronecrotic Mucha-Habermann disease who was cured with methotrexate and review the use of methotrexate for this disorder in the pediatric age group with the relevant literature.

Cerebral Gangliogliomas during Childhood

Neurosurgery ◽  
1983 ◽  
Vol 13 (2) ◽  
pp. 124-128 ◽  
Author(s):  
Leslie N. Sutton ◽  
Roger J. Packer ◽  
Lucy B. Rorke ◽  
Derek A. Bruce ◽  
Luis Schut
Keyword(s):  
Progressive Disease ◽  
Seizure Control ◽  
Symptomatic Improvement ◽  
Age Group ◽  
Increased Intracranial Pressure ◽  
Behavioral Disturbances ◽  
Focal Neurological Deficit ◽  
Pediatric Age Group ◽  
Anticonvulsant Medication

Abstract We have reviewed our experience with gangliogliomas treated in the post-computed tomography (CT) era at the Children's Hospital of Philadelphia. Of 234 newly histologically verified neoplasms seen at our institution since 1975, 10 (4.3%) were gangliogliomas of the cerebral hemispheres. The presenting complaint was seizures in 9 of 10 patients. and in 8 the seizures were poorly controlled despite increasing doses of anticonvulsant medication. At the time of diagnosis, only 2 patients had a focal neurological deficit and none had signs or symptoms of increased intracranial pressure. Learning disability and behavioral disturbances were common in this group of children. The CT appearance of these lesions was characteristic: most appeared as a cerebrospinal fluid density area that was located peripherally and often indented the skull. There was little contrast enhancement, and a few were diagnosed initially as arachnoid or porencephalic cysts. Despite the CT appearance, all but 2 of the lesions were found to be solid at operation. In one patient, the lesion appeared as an enhancing lesion of the thalamus, and this patient died. Operation resulted in symptomatic improvement. Eight of 9 children are alive, with a follow-up of 1 to 80 months (median, 19.5 months), and are free of progressive disease. Five are seizure-free while receiving anticonvulsant therapy and an additional 3 have improved seizure control as a result of operation. Two of the 3 children with intellectual difficulties preoperatively have shown improvement on testing after operation, probably the result of improved seizure control. One patient developed a disseminating malignancy and died. It is concluded that worsening seizures in the pediatric age group should warrant CT examination and that ganglioglioma should be included in the differential diagnosis of low density areas on CT.

scholarly journals Vitreous Hemorrhage in Pediatric Age Group

2014 ◽  
Vol 2014 ◽  
pp. 1-12 ◽  
Author(s):  
Dora H. AlHarkan ◽  
Eman S. Kahtani ◽  
Priscilla W. Gikandi ◽  
Ahmed M. Abu El-Asrar
Keyword(s):  
Visual Acuity ◽  
Retinal Detachment ◽  
Vitreous Hemorrhage ◽  
Visual Outcome ◽  
Age Group ◽  
Pediatric Age ◽  
Pediatric Age Group ◽  
Good Visual Outcome ◽  
Negative Predictor

Purpose.To identify and study causes of vitreous hemorrhage (VH) in pediatric age group and to investigate factors predicting visual and anatomical outcomes.Procedure.A retrospective review of patients aged 16 years or less with the diagnosis of vitreous hemorrhage from January 2005 until December 2010.Results.A total number of 230 patients (240 eyes) were identified. Traumatic vitreous hemorrhage accounted for 82.5%. In cases of accidental trauma, final visual acuity of 20/200 was significantly associated with visual acuity of ≥20/200 at presentation and the absence of retinal detachment at last follow-up. Patients with nontraumatic vitreous hemorrhage were significantly younger with higher rates of enucleation/evisceration/exenteration and retinal detachment at last follow-up compared to traumatic cases.Conclusion.Trauma is the most common cause of VH in pediatric age group. In this group, initial visual acuity was the most important predictor for visual outcome, and the presence of retinal detachment is a negative predictor for final good visual outcome. The outcome is significantly worse in nontraumatic cases compared to traumatic cases.

Pediatric Subtalar Joint Synovial Chondromatosis

2015 ◽  
Vol 105 (5) ◽  
pp. 435-439 ◽  
Author(s):  
Balaji Saibaba ◽  
Pebam Sudesh ◽  
Gokul Govindan ◽  
Mahesh Prakash
Keyword(s):  
Lower Limb ◽  
Subtalar Joint ◽  
Clinical Presentation ◽  
Surgical Excision ◽  
Synovial Chondromatosis ◽  
Age Group ◽  
The Third ◽  
Synovial Osteochondromatosis ◽  
Pediatric Age Group

Synovial chondromatosis is a rare, usually benign disorder affecting the population predominantly in the third and fourth decades of life and mainly involving the large weightbearing joints of the lower limb—the knees and the hip. In this report, we highlight an unusual pediatric clinical presentation of synovial osteochondromatosis involving the subtalar joint and discuss its surgical management; we also provide a comprehensive up-to-date literature review of the disorder. This patient was successfully treated with en masse surgical excision. He has been doing well, with complete pain relief and improved range of motion at 1-year follow-up. An exceptional involvement of the subtalar joint and an unusual presentation in the pediatric age group makes this case unique.

scholarly journals Revisiting ureteral substitution and its outcome in children: single centre 13 year experience

2020 ◽  
Vol 7 (6) ◽  
pp. 1218
Author(s):  
Natasha L. Vageriya ◽  
Shivaji B. Mane ◽  
Taha Daginawala ◽  
Himangi Athawhale ◽  
Hussain Kotawala ◽  
...  
Keyword(s):  
Age Group ◽  
Pediatric Age ◽  
Single Centre ◽  
Renal Functions ◽  
Pediatric Age Group ◽  
Small Series ◽  
Ureteral Replacement ◽  
Paediatric Age ◽  
Published Paper

Background: Objective of this study is to explore various ureteric substitutes in pediatric age group and their outcomes.Methods: Retrospective analysis was done from 2003-2016, of all patients operated in this hospital that had undergone ureteral replacement. Thirteen such patients (5 from initially published paper (1) and 8 new patients) were followed up to find conduit patency, renal function and related complications with their outcomes assessed.Results: Ureteric substitution was done in 8 patients. Age of the patient ranged from 4 months to 8 years. Out of these for 2 patients monti tube was created; one with colon and other jejunum, rest of the 6 patient appendix was used. On follow up one patient had early appendico-ureteral leak requiring re-anastomosis and one patient had partial obstruction at 6 months corrected by dividing mesentry and untwisting appendix. With a mean follow up of 3.8 years all patients have preserved renal functions and drainage present. Also 5 patients of ureteral substitution performed at this institution and published prior were followed up with mean follow up of 10.4 years having no complaints with preserved function and unobstructed drainage.Conclusions: This small series supports that not only appendix and ileum but even colon as well as jejunum should be considered as Monti’s tube for ureteric replacement, when confronting with short ureter in paediatric age group.

scholarly journals Autoimmune pulmonary alveolar proteinosis successfully treated with lung lavage in an adolescent patient: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Abdalla Mohmed Alasiri ◽  
Reem Abdullah Alasbali ◽  
Meaad Ali Alaqil ◽  
Aishah Marei Alahmari ◽  
Nouf Dagash Alshamrani ◽  
...  
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Lung Lavage ◽  
Age Group ◽  
Pediatric Age ◽  
Adolescent Patient ◽  
Low Oxygen ◽  
Whole Lung Lavage ◽  
Pediatric Age Group ◽  
Alveolar Proteinosis

Abstract Background Pulmonary alveolar proteinosis is a rare interstitial lung disease characterized by accumulating surfactant materials in the alveoli. The autoimmune form is by far the most common in adults, while in the pediatric age group, the vast majority of cases are congenital. We report a case of an adolescent patient diagnosed with autoimmune pulmonary alveolar proteinosis, which is unusual in this age group. Case presentation A-15 year-old Saudi male presented to the emergency department with a history of shortness of breath and low oxygen saturation. High-resolution computed tomography of his chest showed a global crazy-paving pattern. Autoantibodies against granulocyte-macrophage colony-stimulating factor were detected in his serum. A diagnosis of the autoimmune form of pulmonary alveolar proteinosis was confirmed after excluding other possible causes. The patient improved after he underwent whole lung lavage under general anesthesia, and he was independent of oxygen therapy after 6 months of follow-up. Conclusion The autoimmune form of pulmonary alveolar proteinosis is rare in the pediatric age group and should be considered when no apparent cause of this disease was found. Whole lung lavage should be the first treatment modality offered in this setting with close follow-up and monitoring.

scholarly journals Primary Corneoscleral Cyst in a Pediatric Patient

2017 ◽  
Vol 8 (2) ◽  
pp. 425-428 ◽  
Author(s):  
Charudutt Kalamkar ◽  
Amrita Mukherjee
Keyword(s):  
Visual Acuity ◽  
Surgical Excision ◽  
Corneal Opacity ◽  
Age Group ◽  
Pediatric Age ◽  
Visual Axis ◽  
Pediatric Age Group ◽  
Eye Examination ◽  
Scleral Graft

Purpose: Primary corneoscleral cyst is a rare disease occurring in the pediatric age group. We report a case of corneoscleral cyst with visual diminution. Methods: We conducted a case report. Results: A 7-year-old girl presented with corneal opacity in the left eye. Examination revealed a corneoscleral cyst. The corneal part of the cyst involved visual axis. Surgical excision with a scleral graft was performed, leading to an improvement in visual acuity. No recurrences were observed until the last follow-up at 18 months. Conclusions: Corneoscleral cyst should be considered in the differential diagnosis of cystic ocular surface disorders in the pediatric age group.

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