scholarly journals Primary Anetoderma and Acquired Cutis Laxa Associated with Glomerulonephritis in a 37-year-old Filipino Male: A Case Report

2021 ◽  
Vol 55 (5) ◽  
Author(s):  
Val Constantine S. Cua ◽  
Eileen Liesl A. Cubillan
Keyword(s):  
Cutis Laxa ◽  
Elastic Fibers ◽  
Type I ◽  
Review Of Literature ◽  
Reticular Dermis ◽  
Pelvic Area ◽  
History Of ◽  
Work Up ◽  
The Complement System

A 37-year-old Filipino man presented with a 9-month history of sagging skin progressing cephalocaudally from the chin and neck to the axillae, side of the trunk, and pelvic area. This was followed by a 2-month history of increasing serum creatinine levels associated with periorbital and bipedal edema, generalized weakness, decreased appetite, vomiting, and headache. Subsequently, skin-colored, non-tender sac-like plaques appeared on the abdomen, inguinal, and intergluteal areas. Histopathology of the latter lesions showed increased spaces between collagen bundles in the dermis. Staining with Verhoeff-van Gieson revealed focal sparse elastic fibers in the papillary dermis compared to that of the reticular dermis consistent with anetoderma. Further work-up revealed normal ANA titer and low serum C3. Kidney biopsy showed IgG deposition in the tubular basement membrane and trace C3 deposition in the glomerular mesangium, giving a diagnosis of rapid progressive glomerulonephritis. On subsequent follow-up, the sac-like plaques became lax and presented as generalized wrinkling of the skin, raising the question whether cutis laxa and anetoderma are occurring in a spectrum instead as distinct entities. Based on the current review of literature, this is the first reported case of primary anetoderma co-occurring with cutis laxa in a patient with glomerulonephritis. Deposition of immunoglobulins along the elastic fibers could have activated the complement system, mediating the destruction of the elastic fibers, resulting to cutis laxa and anetoderma. This case also considers the possibility of anetoderma and type I acquired cutis laxa occurring either in a spectrum or as distinct diseases in a single patient. Further investigations may identify an ultrastructural pattern that can help differentiate the two entities.

scholarly journals Type I split cord malformation and tethered cord syndrome in an adult patient: A case report and literature review

2019 ◽  
Vol 10 ◽  
pp. 90
Author(s):  
Erin N. D’Agostino ◽  
Daniel R. Calnan ◽  
Vyacheslav I. Makler ◽  
Imad Khan ◽  
John H. Kanter ◽  
...  
Keyword(s):  
Conservative Therapy ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Leg Pain ◽  
Split Cord Malformation ◽  
Type I ◽  
Review Of Literature ◽  
History Of ◽  
Magnetic Resonance Imaging Mri

Background: In a split cord malformation (SCM), the spinal cord is divided longitudinally into two distinct hemicords that later rejoin. This can result in a tethered cord syndrome (TCS). Rarely, TCS secondary to SCM presents in adulthood. Here, we present an adult female with Type I SCM resulting in TCS and a review of literature. Case Description: A 57-year-old female with a history of spina bifida occulta presented with a 2-year history of worsening back and left leg pain, difficulty with ambulation, and intermittent urinary incontinence; she had not responded to conservative therapy. Magnetic resonance imaging (MRI) revealed a tethered cord secondary to lumbar type I SCM. The patient underwent an L1–S1 laminectomy for resection of the bony septum with cord detethering. At 2-month follow-up, the patient had improvement in her motor symptoms and less pain. In literature, 25 cases of adult-onset surgically managed SCM with TCS were identified (between 1936 and 2018). Patients averaged 37 years of age at the time of diagnosis, and 56% were female. Conclusion: TCS can present secondary to SCM in adulthood and is characterized predominantly by back and leg pain.

scholarly journals Neonate born with ischemic limb to a COVID-19 positive mother: management and review of literature

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Shahana Perveen ◽  
Karmaine A. Millington ◽  
Suchitra Acharya ◽  
Amit Grag ◽  
Vita Boyar
Keyword(s):  
Differential Diagnosis ◽  
Compartment Syndrome ◽  
Upper Extremity ◽  
Preterm Neonate ◽  
Review Of Literature ◽  
Case Presentation ◽  
Ischemic Limb ◽  
History Of ◽  
Hand Amputation ◽  
Work Up

AbstractObjectivesTo describe challenges in diagnosis and treatment of congenital neonatal gangrene lesions associated with history of maternal coronavirus disease 2019 (COVID-19) infection.Case presentationA preterm neonate was born with upper extremity necrotic lesions and a history of active maternal COVID-19 infection. The etiology of his injury was challenging to deduce, despite extensive hypercoagulability work-up and biopsy of the lesion. Management, including partial forearm salvage and hand amputation is described.ConclusionsNeonatal gangrene has various etiologies, including compartment syndrome and intrauterine thromboembolic phenomena. Maternal COVID-19 can cause intrauterine thrombotic events and need to be considered in a differential diagnosis.

Real-World Experience with Type I Endoleaks after Endovascular Repair of the Thoracic Aorta

2010 ◽  
Vol 76 (6) ◽  
pp. 599-605 ◽  
Author(s):  
Joshua D. Adams ◽  
Margaret C. Tracci ◽  
Sahir Sabri ◽  
Kenneth J. Cherry ◽  
John F. Angle ◽  
...  
Keyword(s):  
Natural History ◽  
Thoracic Endovascular Aortic Repair ◽  
Spontaneous Resolution ◽  
Type I ◽  
Single Center ◽  
Type I Endoleak ◽  
History Of ◽  
Aortic Pathologies ◽  
Initial Results

Endoleaks are a frequent complication of thoracic endovascular aortic repair (TEVAR) and will likely increase in incidence with application of the technique to more complicated aortic anatomy and a wider range of thoracic aortic pathologies. Management generally consists of aggressive repair of Type I endoleaks; however, the natural history of Type I endoleaks after TEVAR remains largely unknown. The purpose of this study was to examine the incidence and characteristics of Type I endoleaks and to evaluate clinical outcomes of patients with Type I endoleaks after TEVAR. A single-center retrospective review was performed on all patients who underwent TEVAR over a 4-year period. Type I endoleaks were detected in 21 per cent (27 of 129) of patients on post-deployment aortography or CT angiography. During a mean follow-up of 750.63 ± 483 days, 59 per cent (16 of 27) closed spontaneously; 30 per cent (eight of 27) required secondary endovascular intervention; and 11 per cent (three of 27) have persisted with no increase in maximum aortic diameter. No patients have died or required open surgical conversion as a result of their Type I endoleak. Although accurate predictors of spontaneous resolution of Type I endoleaks have yet to be definitively characterized, our initial results suggest that it may be safe to observe small Type I endoleaks given that a large percentage resolve spontaneously and no endoleak-related deaths have occurred.

scholarly journals A COMPARISON BETWEEN SYNTHETIC AND BIOSYNTHETIC MESHES IN THE SURGICAL TREATMENT OF SEVERE GENITAL PROLAPSE: RESULTS AND COMPLICATIONS

2010 ◽  
Vol 23 (3) ◽  
pp. 21
Author(s):  
S. Dati ◽  
V. De Lellis ◽  
P. Palermo ◽  
G. Carta
Keyword(s):  
Genital Prolapse ◽  
Pelvic Organ ◽  
Wilcoxon Test ◽  
Type I ◽  
Replacement Surgery ◽  
Group A ◽  
Group B ◽  
Work Up

The effectiveness, tolerability and complications of two surgical procedures using prosthetic materials with different physical and structural properties were assessed with a full Urogynecology work-up, through a retrospective study of 158 patients with severe genital prolapse (POP-Q staging III-IV) selected from November 2006 to April 2009. Eighty-six patients underwent fascial replacement surgery with ProliftTM System with a dual transobturator access in the anterior district and a transperineal posterior access with a synthetic polypropylene type I mesh (Group A). Seventy-two patients who underwent pelvic organ prolapse surgery with Avaulta/Avaulta PlusTM System with a dual transobturator access in the anterior district and a dual transperineal posterior access with a biosynthetic polypropylene type I mesh coated with a film of hydrophilic porcine collagen were placed in Group B. There were no intra and postoperative complications. Results of mean 20.8 month follow-up showed an effective anatomical cure rate of 89.5% in group A and 86.1% in group B and a low percentage of erosive complications, 8.1% and 5.6% respectively. Validated questionnaires for prolapse, the UDI 6 s.f., the IIQ7 s.f. and the PISQ-12 all showed a statistically significant improvement of quality of life in patients undergoing the two procedures (Wilcoxon test: P<0.001).

scholarly journals Idiopathic granulomatous hypophysitis presenting with galactorrhea, headache, and nausea in a woman: a case report and review of the literature

2019 ◽  
Vol 13 (1) ◽  
Author(s):  
Guive Sharifi ◽  
Mohammad Reza Mohajeri-Tehrani ◽  
Behrouz Navabakhsh ◽  
Bagher Larijani ◽  
Touraj Valeh
Keyword(s):  
Pituitary Gland ◽  
Inflammatory Disease ◽  
Transsphenoidal Surgery ◽  
Brain Magnetic Resonance Imaging ◽  
Serum Prolactin ◽  
Granulomatous Hypophysitis ◽  
History Of ◽  
Work Up ◽  
Visual Complaints

Abstract Background Inflammation of the pituitary gland can occur in a variety of primary or secondary disorders. Idiopathic granulomatous hypophysitis is a rare inflammatory disease of the pituitary gland that can closely mimic a pituitary adenoma clinicoradiologically. Most authorities agree on minimally invasive transsphenoidal surgery as the mainstay in diagnosis and treatment of this disorder. There is still some controversy regarding pure medical management of idiopathic granulomatous hypophysitis in the literature. Case presentation A 47-year-old Iranian woman of Azeri ethnicity with a history of benign breast cysts with a chief complaint of galactorrhea presented to our endocrinology clinic. Her past medical history was negative for any menstrual irregularities, hirsutism, visual complaints, diplopia, polyuria and polydipsia or seizures. She was taking 100 mcg of levothyroxine daily. Her familial history and physical examination were unremarkable. Her initial laboratory work-up revealed hyperprolactinemia (82.4 ng/mL) with otherwise normal pituitary axes. Brain magnetic resonance imaging showed a pituitary macroadenoma for which she was treated with 0.5 mg of cabergoline weekly. Although her serum prolactin level dropped to 1.7 ng/mL and her galactorrhea was resolved, she continued to complain of headaches and nausea. Repeated imaging showed no decrease in size of the macroadenoma. Therefore, she underwent transsphenoidal surgery of the macroadenoma which was reported as chronic granulomatous hypophysitis by expert pathologists. Tuberculosis, sarcoidosis, Wegener’s granulomatosis, Langerhans cell histiocytosis, and syphilis were ruled out by appropriate tests and she was diagnosed as having idiopathic granulomatous hypophysitis. Fortunately, her condition was not complicated by hypopituitarism and she was symptom free 9 months after transsphenoidal surgery. Conclusions Idiopathic granulomatous hypophysitis, a rare inflammatory disease of the pituitary gland, is a diagnosis of exclusion for which both medical and surgical management are reported in the literature. We present a case of idiopathic granulomatous hypophysitis who was symptom free with no complications of hypopituitarism following its transsphenoidal resection after 9 months of follow-up.

scholarly journals Unusual presentation of vitamin D3-dependent rickets type II in a kitten

2020 ◽  
Vol 6 (1) ◽  
pp. 205511692091027
Author(s):  
Florent Duplan ◽  
Christina Maunder
Keyword(s):  
Vitamin D ◽  
Positive Response ◽  
Abdominal Distension ◽  
Response To Treatment ◽  
Type I ◽  
Type Ii ◽  
Different Types ◽  
History Of ◽  
Radiographic Changes

Case summary A 15-week-old kitten presented with a 1-month history of intermittent generalised tremors and abdominal distension. Hypocalcaemia associated with increased 1,25-vitamin D3 was consistent with vitamin D3-dependent rickets type II. The bone appearance on CT scan was most consistent with the changes typically seen with nutritional secondary hyperparathyroidism and less typical of the changes seen with rickets. Our patient had a positive response to high vitamin D3 therapy as it remained normocalcaemic 16 months after diagnosis, supporting the diagnosis of rickets. Relevance and novel information This case report is an unusual and interesting presentation of rickets in a kitten. Despite the characteristic vitamin D3 disturbance for rickets type II, the atypical radiographic changes have not been previously reported. In the literature, a positive response to treatment is not commonly seen or follow-up is short. Our case responded well to treatment and was followed for 16 months from the time of diagnosis. This emphasises that the pathophysiology of the condition is not well understood, and that different types of vitamin D3-dependent rickets type II may exist. Although the genetic defects responsible for some cases of rickets type I have been identified, this has still not been determined for rickets type II.

Successful treatment of disseminated granulomatous aspergillosis in an apparently immunocompetent host

2020 ◽  
Vol 50 (4) ◽  
pp. 346-349 ◽  
Author(s):  
Umang Agrawal ◽  
Pratik Savaj ◽  
Kanishka Davda ◽  
Rajeev Soman ◽  
Anjali Shetty ◽  
...  
Keyword(s):  
Granulomatous Inflammation ◽  
Surgical Excision ◽  
Immunocompetent Host ◽  
Short History ◽  
Normal Individuals ◽  
History Of ◽  
One Year ◽  
Work Up ◽  
The Brain

A young Indian man presented elsewhere with a short history of haematuria and cough. Investigations revealed renal and pulmonary lesions. Histopathology of these lesions was reported as mucormycosis. He consulted us two months after onset of symptoms, asymptomatic and clinically well, having received no treatment. In view of clinico-histopathological discordance, a review of the biopsy slides was advised but the patient refused further work-up at that time. One week later, however, he was admitted with left hemiparesis. Brain imaging showed an abscess. He underwent surgical excision of the brain abscess and nephrectomy. Review of previous slides showed septate fungal filaments with granulomatous inflammation. Intraoperative cultures grew Aspergillus flavus. He received voriconazole for one year and is well at his two-year follow-up. His immunological work-up was negative for immunodeficiency. This case illustrates that granulomatous aspergillosis may be an indolent infection in apparently normal individuals and reiterates the importance of interpreting diagnostic reports in conjunction with clinical features.

Resection and primary repair of an inferior vena cava aneurysm in a 13-year-old male

Vascular ◽  
2011 ◽  
Vol 19 (4) ◽  
pp. 218-222 ◽  
Author(s):  
Cynthia Weber ◽  
Keith Jones ◽  
Ross Milner
Keyword(s):  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Primary Repair ◽  
Vena Cava ◽  
Extensive Discussion ◽  
Left Lower Extremity ◽  
History Of ◽  
Large Aneurysm ◽  
Work Up

We present a case of a 13-year-old boy who presented with a three-day history of left lower extremity swelling and a deep venous thrombosis. On further work-up he was discovered to have a large aneurysm of the inferior vena cava. After extensive discussion with his family, he underwent resection and primary repair of the inferior vena cava without complication. At a six-month follow up appointment, he was doing well with no recurrent aneurysmal degeneration on computed tomography scan imaging.

scholarly journals Primary Myelofibrosis Presenting as Extramedullary Hematopoiesis in a Transplanted Liver Graft: Case Report and Review of the Literature

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Ghulam Rehman Mohyuddin ◽  
Abdulraheem Yacoub
Keyword(s):  
Liver Transplant Recipient ◽  
Graft Function ◽  
Primary Myelofibrosis ◽  
Extramedullary Hematopoiesis ◽  
Liver Graft ◽  
Hematologic Neoplasm ◽  
History Of ◽  
Hypercellular Marrow ◽  
Work Up

Primary myelofibrosis (PMF) commonly results in extramedullary hematopoiesis (EMH) in the spleen and liver as well as a variety of other organs. We present a first report of a unique presentation of PMF in a liver transplant recipient patient as EMH in the transplanted liver graft. A 76-year-old man with history of cryptogenic cirrhosis received cadaveric liver transplantation in 1996. He maintained a normal graft function and stable hematologic parameters until 2013 when he presented with anemia and progressive fatigue. Extensive work-up did not identify the etiology of the recent decline in his hemoglobin; thus a liver biopsy was done which showed findings of EMH within the sinusoids with increased megakaryocytes, some with atypical morphology. A BM biopsy revealed a hypercellular marrow, moderately increased reticulin fibrosis, and features consistent with primary myelofibrosis. Abdominal imaging showed a normal-size spleen and did not identify any sites of EMH outside of the liver. The diagnosis of myelofibrosis was thus made, and this case demonstrated predominant tropism to a transplanted liver graft with absence of EMH elsewhere. We would thus like to emphasize that findings of EMH in subjects with no preexisting hematologic neoplasm should warrant close follow-up and assessment.

Sign in / Sign up

Export Citation Format

Share Document