Publication date: Available online 27 November 2018
Source: Anesthesiology Clinics
Author(s): Evie G. Marcolini, Jennifer S. Albrecht, Kinjal N. Sethuraman, Lena M. Napolitano
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Publication date: Available online 27 November 2018
Source: Anesthesiology Clinics
Author(s): Evie G. Marcolini, Jennifer S. Albrecht, Kinjal N. Sethuraman, Lena M. Napolitano
Most criteria require a parathyroid hormone (PTH) decline of ≥50% within 10 minutes after excision of the gland during surgery for primary hyperparathyroidism. The aim was to evaluate a model allowing earlier prediction of cure.
One thousand eighteen patients with primary hyperparathyroidism were included. A ≥50% decline from baseline within 10 minutes after excision intraoperatively predicted complete removal of hypersecreting tissue. The data were reanalyzed regarding a criterion, requiring a ≥50% decline after 5 minutes. Based on the data and the present literature, an algorithm was created.
Assay predicted cure in 854 patients (true‐positive; 83.89%) after 10 minutes (false‐positive [FP] in 13 patients; 1.52%). Nevertheless, only 14 (1.83%) showed persisting disease. According to the "5 minutes" criterion, 723 patients (71.02%) showed a ≥50% decline from baseline within 5 minutes and 10 (1.38%) patients had a FP decline (sensitivity 0.75, specificity 0.86).
Using a modified criterion, surgery can be discontinued 5 minutes earlier in 71%.
This systematic review qualitatively summarizes the current literature on diagnosis and treatment of oropharyngeal dysphagia (OD) after total laryngectomy (TLE).
Electronic databases PubMed, Embase, and the Cochrane Library were used. Two independent reviewers carried out the literature search and assessed the methodological quality of the included studies using a critical appraisal tool.
Forty‐four articles met the inclusion criteria. Of these, 35 studies were on diagnosis, four on therapy, and five on both diagnosis and treatment of OD following TLE. Study aims, swallowing‐assessment methods, and main findings of the included studies were summarized and presented.
The reviewers found heterogeneous outcomes and serious methodological limitations, which prevented us from pooling data to identify trends that would assist in designing best clinical practice protocols for OD following TLE. Further research should focus on several remaining gaps in our knowledge on diagnosis and treatment interventions for OD following TLE.
Alterations in the human epidermal growth factor receptor‐2 (HER2) pathway have been identified in a subset of salivary duct carcinomas. Dual HER2 inhibition with trastuzumab and pertuzumab has superior antitumor efficacy to trastuzumab monotherapy in HER2‐positive breast cancer, yet its efficacy in HER2‐positive salivary duct carcinoma is unknown.
We report 2 cases of exceptional responses of HER2‐positive salivary duct carcinomas to dual HER2 blockade and docetaxel combination and their molecular characteristics.
A 54‐year‐old man with recurrent metastatic HER2 expressing salivary duct carcinoma of the parotid gland after definitive concurrent chemoradiation achieved a complete response (CR) after 6 cycles of trastuzumab, pertuzumab, and docetaxel (TPH). A 42‐year‐old woman with HER2‐positive salivary duct carcinoma of the parotid gland with bone and liver metastases had CR with TPH and remains in remission on maintenance trastuzumab and pertuzumab.
Dual HER2 blockage resulted in CR in patients with HER expressing salivary duct carcinoma and warrants further evaluation in this patient population.
Aneurysmal bone cysts are rare, benign expansile tumors most commonly involving long bones and vertebrae in patients younger than age 20. Skull base involvement is rare. Aneurysmal bone cysts shares radiological and histological features with other bone tumors, posing a diagnostic challenge.
We report the case of a 32‐year‐old man who presented with facial pain, epistaxis, and cranial neuropathies secondary to a massive tumor of the maxillary sinus and anterior skull base. The tumor was originally misdiagnosed as osteosarcoma. However, genomic analysis revealed a rearrangement in the USP6 gene, elucidating a diagnosis of primary aneurysmal bone cysts. The patient was treated with denosumab. Within 5 months, the patient was asymptomatic with CT showing ossification of the tumor.
This case highlights (1) the utility of genomic analysis in aggressive bone tumors when the diagnosis is unclear and (2) the effectiveness of denosumab as a treatment for aneurysmal bone cysts when surgical resection is unfavorable.
Severe congenital neutropenia (SCN) is a rare disease that involves a heterogeneous group of hereditary diseases. Mutations in the HAX1 gene can cause an autosomal recessive form of SCN-characterized low blood neutrophil count from birth, increased susceptibility to recurrent and life-threatening infections, and preleukemia predisposition. A 7-year-old boy was admitted due to life-threatening infections, mental retardation, and severe neutropenia. He had early-onset bacterial infections, and his serial complete blood count showed persistent severe neutropenia. One older sister and one older brother of the patient died at the age of 6 months and 5 months, respectively, because of severe infection. Bone marrow analysis revealed a maturation arrest at the promyelocyte/myelocyte stage with few mature neutrophils. In direct DNA sequencing analysis, we found a novel homozygous frameshift mutation (c.423_424insG, p.Gly143fs) in the HAX1 gene, confirming the diagnosis of SCN. The patient was successfully treated with granulocyte colony-stimulating factor (G-CSF) and antibiotics. A child with early-onset recurrent infections and neutropenia should be considered to be affected with SCN. Genetic analysis is useful to confirm diagnosis. Timely diagnosis and suitable treatment with G-CSF and antibiotics are important to prevent further complication.
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