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Pituitary Jun 2024The success and outcomes of repeat endoscopic transsphenoidal surgery (ETS) for residual or recurrent Cushing's disease (CD) are underreported in the literature. This...
PURPOSE
The success and outcomes of repeat endoscopic transsphenoidal surgery (ETS) for residual or recurrent Cushing's disease (CD) are underreported in the literature. This study aims to address this gap by assessing the safety, feasibility, and efficacy of repeat ETS in these patients.
METHODS
A retrospective analysis was conducted on 56 patients who underwent a total of 65 repeat ETS performed by a single neurosurgeon between January 2006 and December 2020. Data including demographic, clinical, laboratory, radiological, and operative details were collected from electronic medical records. Logistic regression was utilized to identify potential predictors associated with sustained remission.
RESULTS
Among the cases, 40 (61.5%) had previously undergone microscopic surgery, while 25 (38.5%) had prior endoscopic procedures. Remission was achieved in 47 (83.9%) patients after the first repeat ETS, with an additional 9 (16.1%) achieving remission after the second repeat procedure. During an average follow-up period of 97.25 months, the recurrence rate post repeat surgery was 6.38%. Sustained remission was achieved in 48 patients (85.7%), with 44 after the first repeat ETS and 4 following the second repeat ETS. Complications included transient diabetes insipidus (DI) in 5 (7.6%) patients, permanent (DI) in 2 (3%) patients, and one case (1.5%) of panhypopituitarism. Three patients (4.6%) experienced rhinorrhea necessitating reoperation. A serum cortisol level > 5 µg/dL on postoperative day 1 was associated with a reduced likelihood of sustained remission.
CONCLUSION
Repeat ETS is a safe and effective treatment option for residual or recurrent CD with satisfactory remission rates and low rates of complications.
Topics: Humans; Pituitary ACTH Hypersecretion; Male; Female; Adult; Retrospective Studies; Middle Aged; Treatment Outcome; Endoscopy; Feasibility Studies
PubMed: 38748309
DOI: 10.1007/s11102-024-01396-x -
World Neurosurgery May 2024
In Reply to the Letter to the Editor Regarding "Risk Factors Related to Transient Diabetes Insipidus Development Following Transsphenoidal Pituitary Adenoma Resection: A Multicentric Study".
Topics: Humans; Pituitary Neoplasms; Risk Factors; Adenoma; Diabetes Insipidus; Postoperative Complications; Multicenter Studies as Topic; Neurosurgical Procedures
PubMed: 38741317
DOI: 10.1016/j.wneu.2024.01.175 -
World Neurosurgery May 2024
Letter to the Editor Regarding "Risk Factors Related to Transient Diabetes Insipidus Development Following Transsphenoidal Pituitary Adenoma Resection: A Multicentric Study".
Topics: Humans; Pituitary Neoplasms; Adenoma; Risk Factors; Diabetes Insipidus; Postoperative Complications; Multicenter Studies as Topic; Neurosurgical Procedures
PubMed: 38741316
DOI: 10.1016/j.wneu.2023.11.158 -
Genetic Testing and Molecular Biomarkers Jun 2024Wolfram syndrome (WFS) is an autosomal recessive disorder that often leads to diabetes, optic atrophy, and sensorineural hearing loss. The aim of this study was to...
Wolfram syndrome (WFS) is an autosomal recessive disorder that often leads to diabetes, optic atrophy, and sensorineural hearing loss. The aim of this study was to determine the clinical characteristics and the genetic cause of the first two Moroccan families presenting with WFS. The clinical features of five members of two WFS families were evaluated. Whole-exome sequencing was conducted to explore the underlying genetic cause in the affected patients. Two homozygous variants in the gene were identified, each in one of the two families studied: a missense c.1329C>G variant (p.Ser443Arg) and a nonsense mutation c.1113G>A (p.Trp371Ter). These variants affected conserved amino acid residues, segregated well in the two families, and are absent from genetic databases and in controls of Moroccan origin. Bioinformatics analysis classified the two variants as pathogenic by tools and molecular modeling. Our study identified for the first time two variants in Moroccan patients with WFS that extends the mutational spectrum associated with the disease.
Topics: Adolescent; Adult; Child; Female; Humans; Male; Codon, Nonsense; Exome Sequencing; Homozygote; Membrane Proteins; Morocco; Mutation; Mutation, Missense; Pedigree; Wolfram Syndrome; Young Adult
PubMed: 38721948
DOI: 10.1089/gtmb.2023.0550 -
BMC Cancer May 2024Postoperative central diabetes insipidus (CDI) is commonly observed in craniopharyngioma (CP) patients, and the inflammatory response plays an important role in CPs. We...
BACKGROUND
Postoperative central diabetes insipidus (CDI) is commonly observed in craniopharyngioma (CP) patients, and the inflammatory response plays an important role in CPs. We aimed to evaluate the predictive value of preoperative peripheral inflammatory markers and their combinations regarding CDI occurrence in CPs.
METHODS
The clinical data including preoperative peripheral inflammatory markers of 208 CP patients who underwent surgical treatment were retrospectively collected and analyzed. The preoperative peripheral white blood cells (WBC), neutrophils, lymphocytes, monocytes, platelet (PLT), neutrophil-to-lymphocyte ratio (NLR), derived-NLR (dNLR), monocyte-to-lymphocyte ratio (MLR) and PLT-to-lymphocyte ratio (PLR) were assessed in total 208 CP patients and different age and surgical approach CP patient subgroups. Their predictive values were evaluated by the receiver operator characteristic curve analysis.
RESULTS
Preoperative peripheral WBC, neutrophils, NLR, dNLR, MLR, and PLR were positively correlated and lymphocyte was negatively associated with postoperative CDI occurrence in CP patients, especially when WBC ≥ 6.66 × 10/L or lymphocyte ≤ 1.86 × 10/L. Meanwhile, multiple logistic regression analysis showed that WBC > 6.39 × 10/L in the > 18 yrs age patients, WBC > 6.88 × 10/L or lymphocytes ≤ 1.85 × 10/L in the transcranial approach patients were closely associated with the elevated incidence of postoperative CDI. Furthermore, the area under the curve obtained from the receiver operator characteristic curve analysis showed that the best predictors of inflammatory markers were the NLR in total CP patients, the MLR in the ≤ 18 yrs age group and the transsphenoidal group, the NLR in the > 18 yrs age group and the dNLR in the transcranial group. Notably, the combination index NLR + dNLR demonstrated the most valuable predictor in all groups.
CONCLUSIONS
Preoperative peripheral inflammatory markers, especially WBC, lymphocytes and NLR + dNLR, are promising predictors of postoperative CDI in CPs.
Topics: Humans; Craniopharyngioma; Female; Male; Retrospective Studies; Adult; Pituitary Neoplasms; Postoperative Complications; Adolescent; Middle Aged; Child; Young Adult; Diabetes Insipidus, Neurogenic; Neutrophils; Biomarkers; Lymphocytes; Inflammation; Leukocyte Count; Preoperative Period; Child, Preschool; Prognosis; ROC Curve
PubMed: 38720306
DOI: 10.1186/s12885-024-12324-4 -
SAGE Open Medical Case Reports 2024Herein, we report a rare case of invasive nasopharyngeal carcinoma with extension to the pituitary gland misdiagnosed as a pituitary macroadenoma. A 50-year-old woman...
Herein, we report a rare case of invasive nasopharyngeal carcinoma with extension to the pituitary gland misdiagnosed as a pituitary macroadenoma. A 50-year-old woman was referred to our department with a diagnosis of pituitary macroadenoma. She presented with headache, visual disturbances, weakness, nausea, vomiting, and hypoglycemia. Polyuria was not reported. On pituitary magnetic resonance imaging, a large mass was observed to extend from the sella turcica to the sphenoid sinus, optic chiasm, and nasopharynx, leading to the initial diagnosis of an invasive pituitary macroadenoma. Biochemical investigations revealed corticotropin deficiency, secondary hypothyroidism, hypogonadotropic hypogonadism, and moderate hyperprolactinemia. Hormone replacement therapy was initiated. After hydrocortisone initiation, diabetes insipidus was revealed. Subsequent magnetic resonance imaging showed an infiltration of the nasopharynx with an extension to the pituitary gland. An endoscopic biopsy confirmed the diagnosis of undifferentiated nasopharyngeal carcinoma. The patient was referred to the oncology department for chemo and radiotherapy. Invasive nasopharyngeal carcinoma presenting with pituitary extension is very rare. It should be considered in the differential diagnosis of pituitary macroadenoma with hypopituitarism. Proper management of such cases requires a multidisciplinary approach.
PubMed: 38715901
DOI: 10.1177/2050313X241252337 -
Journal of Nephrology May 2024
PubMed: 38709446
DOI: 10.1007/s40620-024-01945-4 -
Cureus Apr 2024Background Treatment of patients with a giant pituitary neuroendocrine tumor (GPitNET) is challenging. Here, we present the methods used for the clinical management of...
Background Treatment of patients with a giant pituitary neuroendocrine tumor (GPitNET) is challenging. Here, we present the methods used for the clinical management of patients who underwent GPitNET resection mainly via endoscopic endonasal surgery along with multimodal support to avoid surgical complications, which can affect the outcomes. Methodology The medical records of 25 patients with a GPitNET who underwent endonasal endoscopic surgery were retrospectively reviewed. Complications were analyzed and factors affecting the extent of resection were evaluated. Results Gross total resection was achieved in six (24%), near-total resection (>90%) in nine (36%), and partial resection in 10 (40%) patients. Multivariate analyses revealed that tumors invading the middle fossa had negative effects on the extent of resection (odds ratio = 0.092, p = 0.047). Postoperative vision improved or normalized in 16 (64%), remained stable in eight (32%), and worsened in one (4%), while a new hormonal deficit was noted in seven (28%) patients. Complications included permanent oculomotor nerve palsy in one (4%) and transient oculomotor palsy in one (4%), apoplexy of the residual tumor resulting in ischemic stroke in one (4%), postoperative cerebrospinal fluid leakage in one (4%), and permanent diabetes insipidus in six (24%) patients. Conclusions For GPitNETs that extend into the middle fossa, our study underscored the difficulties in surgical extraction and the necessity for tailored treatment approaches. To ensure the safest and most complete removal possible, the surgical strategy must be specifically adapted to each case. Additionally, employing a comprehensive support approach is essential to reduce the chance of complications in patients impacted by this condition.
PubMed: 38707178
DOI: 10.7759/cureus.57498 -
Hormone Research in Paediatrics May 2024Stimulated copeptin may provide an alternative to water deprivation testing (WDT) in the evaluation of polyuria-polydipsia syndrome (PPS). Though best studied, arginine...
INTRODUCTION
Stimulated copeptin may provide an alternative to water deprivation testing (WDT) in the evaluation of polyuria-polydipsia syndrome (PPS). Though best studied, arginine stimulation alone produces a modest copeptin response in children. We investigated the effectiveness of the arginine + LevoDopa/Carbidopa stimulation test (ALD-ST) for copeptin.
METHODS
47 healthy short children (controls), 10 children with primary polydipsia, and 10 children with AVP deficiency received arginine hydrochloride (500 mg/kg intravenously over 30 min) and Levodopa/carbidopa (10:1 ratio; 175 mg of
l -Dopa/m2 BSA) orally. Serum copeptin was measured at 0, 60, 90, and 120 min.RESULTS
In controls, ALD-ST increased copeptin from a median of 7.0 pmol/L (IQR 5.0-10.0) to a peak of 44.0 pmol/L (IQR 21.4-181.0) between 60 and 120 min (p < 0.001). Copeptin peak was higher in subjects who experienced nausea or vomiting (57%) than in those who did not (131.0 pmol/L [IQR 42.5-193.8] vs. 22.7 pmol/L [IQR 16.0-33.7], p < 0.001). While subjects with primary polydipsia had similar baseline (8.5 pmol/L [IQR 8.0-11.0]) and stimulated (125.2 pmol/L [IQR 87.6-174.0]) copeptin levels as controls, subjects with AVP deficiency had lower baseline (2.5 pmol/L [IQR 2.0-3.1]) and peak levels (4.6 pmol/L [IQR 2.4-6.0]). A peak copeptin of ≥9.3 pmol/L best predicted absence of complete or partial AVP deficiency with a sensitivity of 100% and specificity of 80%.
CONCLUSIONS
ALD-ST induced a robust peak copeptin in healthy short children and children with primary polydipsia. Nausea/vomiting, a side effect of ALD-ST, amplified the copeptin response. The ALD-ST may be a suitable initial screening test in children with PPS.
PubMed: 38705144
DOI: 10.1159/000539208 -
Archiv Der Pharmazie May 2024Lithium induces nephrogenic diabetes insipidus (NDI) and microcystic chronic kidney disease (CKD). As previous clinical studies suggest that NDI is dose-dependent and...
Lithium induces nephrogenic diabetes insipidus (NDI) and microcystic chronic kidney disease (CKD). As previous clinical studies suggest that NDI is dose-dependent and CKD is time-dependent, we investigated the effect of low exposition to lithium in a long-term experimental rat model. Rats were fed with a normal diet (control group), with the addition of lithium (Li group), or with lithium and amiloride (Li/Ami group) for 6 months, allowing obtaining low plasma lithium concentrations (0.25 ± 0.06 and 0.43 ± 0.16 mmol/L, respectively). Exposition to low concentrations of plasma lithium levels prevented NDI but not microcystic dilations of kidney tubules, which were identified as collecting ducts (CDs) on immunofluorescent staining. Both hypertrophy, characterized by an increase in the ratio of nuclei per tubular area, and microcystic dilations were observed. The ratio between principal cells and intercalated cells was higher in microcystic than in hypertrophied tubules. There was no correlation between AQP2 messenger RNA levels and cellular remodeling of the CD. Additional amiloride treatment in the Li/Ami group did not allow consistent morphometric and cellular composition changes compared to the Li group. Low exposition to lithium prevented overt NDI but not microcystic dilations of the CD, with differential cellular composition in hypertrophied and microcystic CDs, suggesting different underlying cellular mechanisms.
PubMed: 38704748
DOI: 10.1002/ardp.202400063