Remission of Ectopic Cushing Syndrome Secondary to Medullary Thyroid Cancer With Vandetanib and Selpercatinib.

Medullary thyroid cancer (MTC) is a neuroendocrine tumor associated with activating mutations of the rearranged during transfection (RET) proto-oncogene. These tumors may rarely secrete adrenocorticotropin or corticotropin-releasing hormone, resulting in a paraneoplastic ectopic Cushing syndrome (ECS). Paraneoplastic ECS carries a high risk of mortality, and management is difficult due to the lack of response to antiadrenal therapies. We report on a 37-year-old man who was diagnosed with metastatic MTC and reported symptoms of cortisol excess with laboratory testing in keeping with ECS. He began treatment with vandetanib, a multitargeted tyrosine kinase inhibitor, which resulted in decreased tumor burden as well as clinical and biochemical resolution of ECS. Due to progressive structural disease 10 months later, he was switched to the selective RET inhibitor selpercatinib, which was followed by a rapid reduction of cortisol nearing the threshold of adrenal insufficiency. Tumor markers were also improved, and repeat imaging showed decreased tumor burden. Our case highlights the efficacy of tyrosine kinase inhibitors in the management of paraneoplastic ECS. Selective RET inhibitors may emerge as preferred targeted treatment options due to better efficacy and toxicity profiles compared to multitargeted inhibitors. Clinicians should monitor for adrenal insufficiency with the use of selective RET inhibitors.

Potential Mechanisms of the Sparing of Atopic Dermatitis in the Diaper Region: A Scoping Review.

Atopic dermatitis (AD) is a chronic, inflammatory skin condition commonly affecting infants with notable sparing of the diaper region. Though sources anecdotally attribute this sparing to the physical barrier formed by the diaper and the subsequent retention of moisture, urine, sweat and feces, no studies have formally investigated the factors contributing to this sparing phenomenon. We performed a scoping literature review to investigate the factors involved in sparing of AD in the diaper region, namely humidity, scratching, urine, sweat, feces, and microbiome composition. A total of 130 papers met the inclusion criteria, and extracted data were analyzed in an iterative manner. Increased local humidity facilitates protective changes at the cellular level and offsets transepidermal water loss. Exposure to urea from both sweat and urine may contribute to improved moisturization of the skin through its natural humectant properties and ability to modulate gene expression. Introduction of flora in feces contributes to the generation of protective immune responses and outcompetes growth of pathogens such as Staphylococcus aureus. Finally, diapers physically prevent scratching, which directly interrupts the itch-scratch cycle classically implicated in AD. Our study reviews factors that may contribute to the sparing of AD in the diaper region in infants. A limitation to our findings is that the studies reviewed here explore the impacts of these factors on AD broadly, and not explicitly in the diaper region. Additional studies investigating this may further our understanding of AD pathogenesis and contribute to the development of effective therapeutics.

IgG4-related prostatitis manifesting as urinary obstruction in a 28-year-old male.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a systemic lymphoproliferative disorder characterized by elevated serum IgG4 levels and tumefactive lesions that can involve nearly every organ system. Involvement of the prostate is rare but has been reported in limited cases. CASE PRESENTATION: A 28-year-old man of Asian descent with a history of sinusitis and priapism presented to hospital with rigors and voiding symptoms. He was diagnosed with IgG4-RD one month prior to presentation, following pathological analysis of a submandibular mass that demonstrated chronic sclerosing sialadenitis. On presentation, white blood cell count, C-reactive protein, and prostate serum antigen levels were all within normal limits. Examination was notable for a large, firm prostate, and a foley catheter was inserted. Contrast CT of the abdomen was unremarkable. Further workup revealed elevated serum IgG4 levels (9.22 g/L) and he was subsequently started on prednisone 35 mg daily. Imaging to screen for systemic IgG4-RD involvement demonstrated paravertebral soft tissue involvement and he was given rituximab 1000 mg IV × 2 doses. MRI revealed diffuse prostatitis. Five days after starting prednisone and one day after his first dose of rituximab, he successfully passed trial of void and was discharged home. CONCLUSIONS: IgG4-related prostatitis is a rare and underrecognized manifestation of IgG4-RD. Our case highlights the need to consider IgG4-related prostatitis as an etiology of urinary obstruction in young individuals. Resolution of symptoms following treatment with steroids may be diagnostic of IgG4-related prostatitis, and may potentially avoid the need for invasive diagnostic procedures such as prostate biopsy.

Pseudo-pheochromocytoma due to obstructive sleep apnea: a case report.

SUMMARY: Obstructive sleep apnea (OSA) is a condition of intermittent nocturnal upper airway obstruction. OSA increases sympathetic drive which may result in clinical and biochemical features suggestive of pheochromocytoma. We present the case of a 65-year-old male with a 2.9-cm left adrenal incidentaloma on CT, hypertension, symptoms of headache, anxiety and diaphoresis, and persistently elevated 24-h urine norepinephrine (initially 818 nmol/day (89-470)) and normetanephrine (initially 11.2 µmol/day (0.6-2.7)). He was started on prazosin and underwent left adrenalectomy. Pathology revealed an adrenal corticoadenoma with no evidence of pheochromocytoma. Over the next 2 years, urine norepinephrine and normetanephrine remained significantly elevated with no MIBG avid disease. Years later, he was diagnosed with severe OSA and treated with continuous positive airway pressure. Urine testing done once OSA was well controlled revealed complete normalization of urine norepinephrine and normetanephrine with substantial symptom improvement. It was concluded that the patient never had a pheochromocytoma but rather an adrenal adenoma with biochemistry and symptoms suggestive of pheochromocytoma due to untreated severe OSA. Pseudo-pheochromocytoma is a rare presentation of OSA and should be considered on the differential of elevated urine catecholamines and metanephrines in the right clinical setting. LEARNING POINTS: Obstructive sleep apnea (OSA) is a common condition among adults. OSA may rarely present as pseudo-pheochromocytoma with symptoms of pallor, palpitations, perspiration, headache, or anxiety. OSA should be considered on the differential of elevated urine catecholamines and metanephrines, especially in patients with negative metaiodobenzylguanidine (MIBG) scan results.

Transmission of Onychomycosis and Dermatophytosis between Household Members: A Scoping Review.

Onychomycosis is a common fungal infection of the nail, caused by dermatophytes, non-dermatophytes, and yeasts. Predisposing factors include older age, trauma, diabetes, immunosuppression, and previous history of nail psoriasis or tinea pedis. Though many biological risk factors have been well characterized, the role of the environment has been less clear. Studies have found evidence of transmission in 44% to 47% of households with at least one affected individual, but the underlying mechanisms and risk factors for transmission of onychomycosis between household members are incompletely understood. A scoping literature review was performed to characterize and summarize environmental risk factors involved in the transmission of onychomycosis within households. A total of 90 papers met the inclusion criteria, and extracted data was analyzed in an iterative manner. Shared household surfaces may harbor dermatophytes and provide sources for infection. Shared household equipment, including footwear, bedding, and nail tools, may transmit dermatophytes. The persistence of dermatophytes on household cleaning supplies, linen, and pets may serve as lasting sources of infection. Based on these findings, we provide recommendations that aim to interrupt household transmission of onychomycosis. Further investigation of the specific mechanisms behind household spread is needed to break the cycle of transmission, reducing the physical and social impacts of onychomycosis.

Management of hypertrophic scars in adults: A systematic review and meta-analysis.

Hypertrophic scars (HTS) are elevated scars which occur due to abnormalities in wound healing after injury and may be associated with pain, pruritus and functional impairment. Despite multiple available treatment options, there is no universal approach to treating HTS. We searched the Web of Science (Core Collection), MEDLINE and EMBASE databases. Title, abstract and full-text screening, along with data extraction, were performed in duplicate. Risk of bias was assessed using the Cochrane risk-of-bias tool. The Vancouver Scar Scale (VSS) scores and mean differences were used for meta-analysis. We screened 3800 abstracts and included 34 randomised controlled trials evaluating treatments for HTS in adults. Silicone and laser modalities improved VSS scores by 5.06 (95% CI: 6.78, 3.34) and 3.56 (95% CI: 5.58, 1.54), respectively. Intralesional triamcinolone combined with silicone or 5-fluorouracil was superior to intralesional triamcinolone monotherapy. Limitations of this study include exclusion of studies which did not utilise VSS, and pooling of studies based on common modalities. Further studies are needed to examine the efficacy of existing and emerging treatment modalities for HTS. Our study supports the treatment of HTS in adults with silicone gel or sheets, injected triamcinolone (preferably combined with 5-fluorouracil or silicone products), pulsed dye laser and fractionated CO2 laser.

The Power of Small Conversations: Bridging the Gap Between Diabetes and Pregnancy Planning.

Despite the established importance of preconception counselling among women with pre-existing diabetes, many Canadian women of this demographic continue to report inadequate medical planning of pregnancy. Primarily due to the teratogenic effects of hyperglycemia in the early weeks of pregnancy, minimizing the risk of adverse pregnancy outcomes requires a proactive approach toward medical optimization before conception. Primary care providers are well placed to provide preconception counselling to reproductive age women with pre-existing diabetes to alert them to the importance of pregnancy planning. This counselling may not necessarily require dedicated visits but may take the form of simple check-ins and key messages interspaced between other interactions. Herein we discuss the importance and challenges of preconception counselling among women with pre-existing diabetes and provide a pragmatic approach to the delivery of preconception counselling among primary care providers.