scholarly journals Pseudohypoaldosteronism Type 1—An Exercise in Clinical Deduction and Critical Management

2017 ◽  
Vol 07 (01) ◽  
pp. e127-e129
Author(s):  
Meenakshi Girish ◽  
Pradeep Pazare ◽  
Archana Jaiswal ◽  
Yash Banait ◽  
Richa Kumar

AbstractPseudohypoaldosteronism type 1 (PHA1) is a life-threatening disorder for two reasons, first because it causes severe hyperkalemia and second because the rarity of the disorder means that diagnosis is often delayed due to the lack of clinician familiarity with this condition. In this case report, we have described how even to an unsuspecting mind and eye, a systematic approach can lead to reversal of the severe hyperkalemia and arrive at the diagnosis of PHA1 as a cause of hyperkalemia. Long-term management can be successful only with dedicated care, and the prognosis is unfortunately worsened by the lack of availability of sodium-K resin in many countries, including India.

Author(s):  
Sudeep K Rajpoot ◽  
Carlos Maggi ◽  
Amrit Bhangoo

Summary Neonatal hyperkalemia and hyponatremia are medical conditions that require an emergent diagnosis and treatment to avoid morbidity and mortality. Here, we describe the case of a 10-day-old female baby presenting with life-threatening hyperkalemia, hyponatremia, and metabolic acidosis diagnosed as autosomal dominant pseudohypoaldosteronism type 1 (PHA1). This report aims to recognize that PHA1 may present with a life-threatening arrhythmia due to severe hyperkalemia and describes the management of such cases in neonates. Learning points PHA1 may present with a life-threatening arrhythmia. Presentation of PHA can be confused with congenital adrenal hyperplasia. Timing and appropriate medical management in the critical care unit prevented fatality from severe neonatal PHA.


2005 ◽  
Vol 43 (05) ◽  
Author(s):  
R Schwab ◽  
P Lakatos ◽  
E Schäfer ◽  
J Weltner ◽  
A Sáfrány ◽  
...  

2015 ◽  
Vol 3 (1) ◽  
pp. 36-47
Author(s):  
Nazma Akter ◽  
Nazmul Kabir Qureshi

Adrenal insufficiency is caused by either primary adrenal failure or by hypothalamic-pituitary impairment of the corticotropic axis. Adrenal insufficiency, first codified in 1855 by Thomas Addison, remains relevant in 2014 because of its lethal nature. Though, it is a rare disease but is life threatening when overlooked. Main presenting symptoms such as fatigue, anorexia and weight loss are nonspecific, thus diagnosis is often delayed. The diagnostic work-up is well established but some pitfalls remain. The diagnosis is adequately established by the 250 ?g ACTH (adrenocorticotropic hormone) stimulation test in most cases. Glucocorticoids provide life saving treatment but long-term quality of life is impaired, perhaps because therapy is not given in a physiologic way. Dehydroepiandrosterone-replacement therapy has been introduced that could help to restore quality of life. It may be useful in pubertal girls, but not in adults. Monitoring of glucocorticoid-replacement is difficult due to lack of objective methods of assessment and is therefore largely based on clinical grounds. Thus, long-term management of patients with adrenal insufficiency remains a challenge, requiring an experienced specialist. DOI: http://dx.doi.org/10.3329/dmcj.v3i1.22238 Delta Med Col J. Jan 2015; 3(1): 36-47


2018 ◽  
Vol 6 ◽  
pp. 2050313X1879504 ◽  
Author(s):  
Matthew Howard ◽  
Anthony Hall

Topical corticosteroids are currently recommended only for short-term management of flares of lichen sclerosus, with efficacy in halting disease progression. Given the chronic nature of this condition, there is a lack of literature surrounding the chronic effects of topical corticosteroids on the male genitalia with many dermatologists avoiding prescribing long term. This case report aims to provide anecdotal observation for the long-term use of topical corticosteroids and details the long-term follow-up of an individual who used potent and superpotent topical corticosteroids for over 25 years without significant demonstrable side effects. A short review on relevant literature is provided.


2010 ◽  
Vol 16 (5) ◽  
pp. 829-834 ◽  
Author(s):  
Oscar Bruno ◽  
Karina Danilowicz ◽  
Marcos Manavela ◽  
Daniela Mana ◽  
Maria Rossi

2021 ◽  
Vol 7 (1) ◽  
pp. 61-66
Author(s):  
Youssef Fahde ◽  
◽  
Davis Mpando ◽  
Mehdi Laghmari ◽  
Houssine Ghannane ◽  
...  

Background and Importance: Transorbitocranial assaults with sharp objects like a knife are rare neuro-ophthalmologic emergencies. However, they can have dramatic functional and life-threatening consequences. Our presentation aims to report the importance of an urgent multidisciplinary approach and to raise awareness among the general population on the importance of preventing violent behavior. Case Presentation: A 33-year-old man was a victim of a knife attack without obvious brain or ophthalmological lesions. The knife entered the medial part of the orbit. Neurological examination was normal, and Computed Tomography (CT) scan showed intracranial trajectory through the orbit to the frontal horn of the lateral ventricle. The knife was extracted without complications. The patient reported spectacular improvement in visual acuity without neurological or oculomotor deficit at long-term follow-up. In this case report, we will discuss the radiological diagnosis and surgical management of transorbital and orbitocranial injuries by foreign body penetration. Conclusion: Urgent multidisciplinary management in orbitocranial trauma by stabbing is mandatory to avoid life-threatening complications and irreversible damages.


2018 ◽  
Vol 5 (1) ◽  
pp. 30
Author(s):  
Majd Qasum ◽  
Samuel N. Heyman ◽  
Jasmin Khateeb ◽  
Muhammad Abu-Arisha ◽  
Said Darawshi ◽  
...  

Metabolic complications, including hyponatremia and metabolic acidosis have been reported following urinary diversion operations, occasionally together with hyper- or hypokalemia, depending on the bowel segment used. While Hypokalemia often accompanies ureterosigmoidostomy and may develop following ileal conduits, we report a rare case of recurrent life threatening hyperkalemia following this procedure, associated with hyponatremia and acidosis. Reviewing the English literature (1973 to 2016) we found 25 cases of hyperkalemia complicating ureteral diversion procedures, mostly after jejunal conduits. Only five cases of hyperkalemia were described after ileal conduit surgeries, including the current report. We discuss the nature of the metabolic and electrolyte disturbances following urinary diversion and debate possible reasons for the rare cases of hyperkalemia complicating ileal conduits.


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