A Case of Neonatal Severe Hyperparathyroidism: Challenges in Management

Neonatal severe hyperparathyroidism is a rare disorder arising from inherited defects in the calcium sensing receptor (CaSR) that presents early in life with severe hypercalcemia, failure to thrive, and developmental retardation. The authors describe an infant with neonatal severe hyperparathyroidism due to homozygous CaSR gene mutation presenting with recurrent episodes of severe hypercalcemia, growth retardation, and developmental delay. Medical management served as an efective bridge therapy to surgery. Total parathyroidectomy with right hemithyroidectomy was performed at 7 mo of age and resulted in successful cure and normalization of growth and developmental milestones. Timely medical and surgical management can help prevent mortality and morbidity in the form of neurodevelopmental sequelae. Life-long monitoring and treatment is mandatory for the resultant hypoparathyroidism.

Exploration on total parathyroidectomy for secondary hyperparathyroidism / 中华耳鼻咽喉头颈外科杂志

To evaluate the clinical significance of dissection parathyroidectomy for secondary hyperparathyroidism (SHPT) in patients with renal disease on maintenance dialysis. We retrospectively reviewed 195 patients with SHPT treated in the Department of Otolaryngology & Head and Neck Surgery of Beijing Civil Aviation General Hospital between September 2009 and September 2017, including 92 males and 103 females, aged from 23 to 77 years old. There were 167 patients by operated firstly and 28 patients by operated secondly for persistent or recurrent SHPT after operation. All patients received dissection parathyroidectomy with parathyroid autograft in the sternocleidomastoid. The easement of symptoms, the levels of serum intact parathyroid hormone (iPTH), serum-ionized calcium, phosphorus, and hemoglobin were compared before and after operation. Data were analyzed by SPSS 22.0 software. Confirmed by postoperative pathology, a total of 804 hyperplastic parathyroid glands were removed in 195 patients with SHPT. Among them, 765 parathyroid glands were clearly identified and located with naked eye. The anatomic distribution of the glands showed 577 (75.4%) in the tracheoesophageal groove. The incidence of ectopic parathyroid glands was 24.6% (188/765). Other 39 (4.9%) hyperplastic parathyroid glands from 22(11.3%) patients, which were not identified and located with naked eye during operation, were pathologically detected in the dissected tissue specimens. Among 195 patients, 28(14.4%) showed supernumerary parathyroid glands. No serious complications occurred after operation. Within 6 months after the operation, the bone pain and skin itch symptoms were completely relieved and, also, the symptoms of muscle weakness, restless leg, anemia and poor sleep quality were significantly alleviated. Following-up at 6 months after surgery showed the serum levels of iPTH [(70.31±60.12) pg/ml], calium [(2.13±0.22) mmol/L], and phosphorus [(1.17±0.27) mmol/L] decreased significantly respectively compared with the preoperative serum levels of iPTH [(1 501.02±167.26) pg/ml], calium [(2.40±0.32) mmol/L], and phosphorus[(2.27±0.50)mmol/L], all with statistically significant differences (0.01); the levels of hemoglobin [(120.32±10.63) g/L] and hematocrit [(39.20±3.21)%] were higher than the preoperative levels of hemoglobin[(104.11±15.17) g/L] and hematocrit [(31.25±5.12)%], both with statistically significant differences ( valve was 12.22,18,37,respectively, all 0.05). Dissection parathyroidectomy is a beneficial and safe surgical procedure for patients with medically refractory SHPT.

Exploration on total parathyroidectomy for secondary hyperparathyroidism / 中华耳鼻咽喉头颈外科杂志

Objective@#To evaluate the clinical significance of dissection parathyroidectomy for secondary hyperparathyroidism (SHPT) in patients with renal disease on maintenance dialysis.@*Methods@#We retrospectively reviewed 195 patients with SHPT treated in the Department of Otolaryngology & Head and Neck Surgery of Beijing Civil Aviation General Hospital between September 2009 and September 2017, including 92 males and 103 females, aged from 23 to 77 years old. There were 167 patients by operated firstly and 28 patients by operated secondly for persistent or recurrent SHPT after operation. All patients received dissection parathyroidectomy with parathyroid autograft in the sternocleidomastoid. The easement of symptoms, the levels of serum intact parathyroid hormone (iPTH), serum-ionized calcium, phosphorus, and hemoglobin were compared before and after operation. Data were analyzed by SPSS 22.0 software.@*Results@#Confirmed by postoperative pathology, a total of 804 hyperplastic parathyroid glands were removed in 195 patients with SHPT. Among them, 765 parathyroid glands were clearly identified and located with naked eye. The anatomic distribution of the glands showed 577 (75.4%) in the tracheoesophageal groove. The incidence of ectopic parathyroid glands was 24.6% (188/765). Other 39 (4.9%) hyperplastic parathyroid glands from 22(11.3%) patients, which were not identified and located with naked eye during operation, were pathologically detected in the dissected tissue specimens. Among 195 patients, 28(14.4%) showed supernumerary parathyroid glands. No serious complications occurred after operation. Within 6 months after the operation, the bone pain and skin itch symptoms were completely relieved and, also, the symptoms of muscle weakness, restless leg, anemia and poor sleep quality were significantly alleviated. Following-up at 6 months after surgery showed the serum levels of iPTH [(70.31±60.12) pg/ml], calium [(2.13±0.22) mmol/L], and phosphorus [(1.17±0.27) mmol/L] decreased significantly respectively compared with the preoperative serum levels of iPTH [(1 501.02±167.26) pg/ml], calium [(2.40±0.32) mmol/L], and phosphorus[(2.27±0.50)mmol/L], all with statistically significant differences (P<0.01); the levels of hemoglobin [(120.32±10.63) g/L] and hematocrit [(39.20±3.21)%] were higher than the preoperative levels of hemoglobin[(104.11±15.17) g/L] and hematocrit [(31.25±5.12)%], both with statistically significant differences (t valve was 12.22,18,37,respectively, all P<0.05).@*Conclusions@#Dissection parathyroidectomy is a beneficial and safe surgical procedure for patients with medically refractory SHPT.

The initial exploration of near-infrared fluorescence-guided total parathyroidectomy in patients with secondary hyperparathyroidism using indocyanine green / 中华内分泌外科杂志

Objective To explore the method and application value of near-infrared fluorescence-guided total parathyroidectomy (TPTX) in patients with secondary hyperparathroidism (SHPT) using indocyanine green (ICG).Methods The clinical data of 38 patients with SHPT undergoing fluorescence-guided TPTX from Dec.2013 to Jun.2017 were collected.All patients were treated with intravenous injection of ICG 30 minutes before operation.Near-infrared fluorescence imaging of parathyroid glands during surgery was observed and all parathyroid glands were resected.The preoperative and postoperative levels of parathyroid hormone,serum calcium and serum phosphorus were recorded to assess total resection and surgical efficacy.The complications such as hemorrhage and wound infection were observed.Results The fluorescence-guided TPTX was successful in all patients.The operation time was 75 to 225 mins.A total of 149 parathyroid glands were localized by fluorescence imaging,including 11 escaped glands by preoperative imaging examination.However,another 7 fluorescence-labeled tissue was inflammatory lymph node or fibrous adipose tissue.Intraoperative fluorescence-guided technique could identify the location and number of parathyroid glands.The level of parathyroid hormone before operation and 30 minutes after resection was (1797.3±838.8) pg/ml and (187.2±109.2) pg/ml,respectively,and maintained low level after operation.The differences were statistically significant(F=155.001,P=0.000).Compared with the level before surgery,the postoperative level of serum calcium and serum phosphorus decreased to normal range with significant differences (F=41.842,P=0.000;F=49.491,P=0.000).No perioperative death happened.One patient sufferred neck hematoma due to heparin,and one patient developed hypertension.No complications such as wound infection and pulmonary infection occurred to any patient.Conclusions Intraoperative fluorescence-guided TPTX using ICG in the patient with SHPT is safet and feasible,and is expected to be a novel technique for intraoperative dynamic examination and real-time localization of parathyroid gland.

Application of total parathyroidectomy with autotransplantation in secondary hyperparathyroidism / 中华内分泌外科杂志

Objective To evaluate the clinical safety and effectiveness of total parathyroidectomy with autotransplantation on the chronic renal failure (CRF) patients who are suffering from severe secondary hyperparathyroidism (SHPT).Methods A retrospective analysis was performed on 149 patients with secondary hyperparathyroidism who were given total parathyroidectomy with autotransplantation from Apr.2010 to Oct.2015.The changes of clinical symptoms,parathyroid hormone,calcium and phosphate blood levels were followed up during 6 months to 6 years.Results 598 parathyroid glands were obtained form 149 patients who underwent surgical resection.Musculoskeletal pain and skin itching relieved or disappeared in 145 cases the 2nd day after operation,while these symptoms gradually relieved one week after operation for 4 cases.Serum intact parathyroid hormone (iPTH)was 89.67±180.61,serum phosphate 1.74±0.52,and serum calcium 2.07±0.32 the 1st day after operation,and they all decreased compared with those before operation(P＜0.001).Serum calcium,phosphorus and iPTH levels were similar at 6,36 and 60 months after operation (P＞0.05).Two patients had pathological fracture after operation.No persistent bone pain or skin itching was found during the follow-up period.Two patients had secondary hyperparathyroidism.Six patients had recurrence,among whom five underwent surgery again,and one patient had clinical follow-up.One patient died of pulmonary infection at 13 months after operation.Conclusion Total parathyroidectomy with autotransplantation were applied to ineffective medical treatment for advanced secondary hyperparathyroidism in patients with chronic renal failure.

Perioperative nursing care of 25 patients underwent total parathyroidectomy with forearm autograft / 中华护理杂志

To summarize key points of nursing care of 25 patients with secondary hyperparathyroidism underwent parathyroid autotransplantation with forearm (tPTX+AT).The integrated mode was adopted during the perioperative period,focusing on the following key points:perioperative management of environment,nursing of vascular access,intro-operative management of sample,nursing management during anesthesia and recovery,management of drawing blood,treatment and nursing care of postoperative hypocalcemia,prevention and nursing care of infection and hemorrhage,wound care in the forearm and monitoring of graft.Compared with the preoperative period,levels of intact parathyroid hormone(iPTH),calcium and phosphorus were significantly decreased during the postoperative period (P＜0.01);symptoms were significantly improved after surgery included bone and joint pain,muscular weakness,and insomnia.

Application of total parathyroidectomy without autotransplantation in refractory secondary hyperparathyroidism / 中国现代普通外科进展

Objective:To analyze the clinical outcome and feasibility for patients who underwent total parathyroidectomy without autotransplantation (TPTX) for secondary hyperparathyroidism (SHPT).Methods:From April 2012 to December 2015,220 SHPT patients underwent TPTX in the department of Breast and Thyroid Surgery of China-Japan Friendship Hospital.The clinical data and effect were assessed retrospectively.Results:All the 220 patients were on permanent dialysis with mean duration of dialysis (7.93 ± 3.75) years.A durable reduction in mean PTH,Ca and P were observed after TPTX (P＜0.01).The mean hospital stay was (7.8 ± 2.8) days.TPTX produced a rapid improvement in clinical symptoms.Incidence of hypocalcemia was 73.46％.Severe complications such as recurrent laryngeal nerve palsy or inactive dynamic osteopathia,haven't been observed postoperatively.The rate of persistent status (PTH≥300 pg/mL) was 9.1％.One (0.45％) died of infectious shock perioperatively.Conclusions:TPTX was a safe and feasible surgical procedure for patients with SHPT.It was worth of being applied.Not missing the parathyroid during operation was the key point for successful TPTX.Intensive monitoring and maintaining stable normocalcemia were the key point to reduce complication.

Application of carbon nanoparticles suspension injection in uremic patients with secondary hyperparathyroidism underwent total parathyroidectomy: 2 case report and literature review / 中南大学学报(医学版)

Total parathyroidectomy (PTX) can be used to treat secondary hyperparathyroidism (SHPT)in uremic patients,relieving the patient's osteolytic destruction.Complete resection of bilateral parathyroid glands is key to successful operation.Recently,2 patients successfully received surgical treatment,in which carbon nanoparticles suspension injection were used.With the help of carbon nanoparticles suspension injection,all of the 4 hyperplastic parathyroid glands were found successfully.The parathyroid hormone(PTH) was significantly decreased after operations and the clinical symptoms were relieved simultaneously.We conclude that the use of carbon nanoparticles suspension injection is helpful for total parathyroidectomy operation in uremic patients with SHPT,which can protect the normal thyroid and recurrent laryngeal nerve.

Comparison of short term curative effects between operation and oral cinacalcet for treating refractory secondary hyperparathyroidism / 重庆医学

Objective To study the short-term curative effect of total parathyroidectomy (t-PTX) and cinacalcet therapy for treating refractory secondary hyperparathyroidism (SHPT).Methods The clinical data of 118 patients with refractory SHPT admitted in our hospital from January 2012 to September 2015 wereretrospectively analyzed,and according to different therapeutic methods the patients were divided into the group A (receiving the conventional medical treatment after poor effect by the impact therapy,n=62),B(performing t-PTX,n=26) and C (oral cinacalcet,n=30).All patients were given the conventional medical treatment.The calcium,phosphorus and calcium-phosphorus product,and parathyroid hormone (iPTH) follow-up on 0,1 d,and at 1,3,6 months was finished in 3 groups.Results The gender,age,dialysis time,calcium,phosphorus,calcium-phosphorus product and iPTH level on 1 d had no statistically significant difference among 3 groups(P＞0.05).the pain relief rates of the group A,B and C were 0,92.31 ％ and 60.00 ％ respectively;compared with the group A,phosphorus,calcium-phosphorus product and iPTH at various time points after processing in the group B and C were significantly decreased(P＜0.01);compared with the group C,calcium,phosphorus,calcium-phosphorus product and iPTH at various time points after processing in the group B were significantly reduced(P＜0.05).Conclusion If the patients with refractory SHPT are tolerable for surgery,t-PTX therapy should be taken as the first choice;oral cinacalcet treatment provides the possibility of non-operative therapy for the patients with refractory SHPT.

Intramuscular and subcutaneous forearm parathyroid autotransplantation after total parathyroidectomy for renal hyperparathyroidism patients / 中华内分泌外科杂志

Objective To compare the effects of intramuscular or subcutaneous forearm parathyroid autotransplantation after total parathyroidectomy on patients with renal hyperparathyroidism.Methods From Jan.2012 to Dec.2013,total parathyroidectomy was carried out in 90 patients with renal hyperparathyroidism.According to the location of their parathyroid autograft,patients were divided into intramuscular group (n =39)and subcutaneous group(n =51).One patient in the intramuscular group and two in the subcutaneous group were excluded for the high levels of intact parathyroid hormone (iPTH) the day after operation.iPHT was analyzed 2 weeks,1 month,3 and 6 months after surgery.Results The time of autotransplantation was significantly shorter in subcutaneous group than in intramuscular group (11.46 (2.63) min vs 22.12 (3.78) min;t =0.632,P ＜ 0.05).iPTH levels were significantly lower in subcutaneous group than in intramuscular group 2 weeks after operation (P ＜ 0.05).There was no significant difference between the 2 groups regarding iPTH levels at 1 month,3 or 6 months after surgery.In the follow-up one patient in intramuscular group and one in subcutaneous group had graft-dependent hyperparathyroidism.Conclusion As compared to intramuscular parathyroid autotransplantation,subcutaneous parathyroid autotransplantation has advantages of simpler to operate,shorter autoimplantation time and easier to autograftectomy.

Primary hyperparathyroidism in multiple endocrine neoplasia type 1: when to perform surgery?

Primary hyperparathyroidism is a common endocrinological disorder. In rare circumstances, it is associated with familial syndromes, such as multiple endocrine neoplasia type 1. This syndrome is caused by a germline mutation in the multiple endocrine neoplasia type 1 gene encoding the tumor-suppressor protein menin. Usually, primary hyperparathyroidism is the initial clinical expression in carriers of multiple endocrine neoplasia type 1 mutations, occurring in more than 90% of patients and appearing at a young age (20-25 years). Multiple endocrine neoplasia type 1/primary hyperparathyroidism is generally accompanied by multiglandular disease, clinically manifesting with hypercalcemia, although it can remain asymptomatic for a long time and consequently not always be recognized early. Surgery is the recommended treatment. The goal of this short review is to discuss the timing of surgery in patients when primary hyperparathyroidism is associated with multiple endocrine neoplasia type 1.

Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy

Usually, primary hyperparathyroidism is the first endocrinopathy to be diagnosed in patients with multiple endocrine neoplasia type 1, and is also the most common one. The timing of the surgery and strategy in multiple endocrine neoplasia type 1/hyperparathyroidism are still under debate. The aims of surgery are to: 1) correct hypercalcemia, thus preventing persistent or recurrent hyperparathyroidism; 2) avoid persistent hypoparathyroidism; and 3) facilitate the surgical treatment of possible recurrences. Currently, two types of surgical approach are indicated: 1) subtotal parathyroidectomy with removal of at least 3-3 K glands; and 2) total parathyroidectomy with grafting of autologous parathyroid tissue. Transcervical thymectomy must be performed with both of these procedures. Unsuccessful surgical treatment of hyperparathyroidism is more frequently observed in multiple endocrine neoplasia type 1 than in sporadic hyperparathyroidism. The recurrence rate is strongly influenced by: 1) the lack of a pre-operative multiple endocrine neoplasia type 1 diagnosis; 2) the surgeon's experience; 3) the timing of surgery; 4) the possibility of performing intra-operative confirmation (histologic examination, rapid parathyroid hormone assay) of the curative potential of the surgical procedure; and, 5) the surgical strategy. Persistent hyperparathyroidism seems to be more frequent after subtotal parathyroidectomy than after total parathyroidectomy with autologous graft of parathyroid tissue. Conversely, recurrent hyperparathyroidism has a similar frequency in the two surgical strategies. To plan further operations, it is very helpful to know all the available data about previous surgery and to undertake accurate identification of the site of recurrence.

Post-surgical follow-up of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1

The bone mineral density increments in patients with sporadic primary hyperparathyroidism after parathyroidectomy have been studied by several investigators, but few have investigated this topic in primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Further, as far as we know, only two studies have consistently evaluated bone mineral density values after parathyroidectomy in cases of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Here we revised the impact of parathyroidectomy (particularly total parathyroidectomy followed by autologous parathyroid implant into the forearm) on bone mineral density values in patients with primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Significant increases in bone mineral density in the lumbar spine and femoral neck values were found, although no short-term (15 months) improvement in bone mineral density at the proximal third of the distal radius was observed. Additionally, short-term and medium-term calcium and parathyroid hormone values after parathyroidectomy in patients with primary hyperparathyroidism associated with multiple endocrine neoplasia type 1 are discussed. In most cases, this surgical approach was able to restore normal calcium/parathyroid hormone levels and ultimately lead to discontinuation of calcium and calcitriol supplementation.

A Case of Surgical Experience of Secondary Hyperparathyroidism / 대한내분비외과학회지

The enlarged parathyroid glands associated with chronic renal failure were recognized during the 1930's. The number of patients on long-term hemodialysis due to chronic renal failure is steadily increasing and the hyperparathyroid state certainly became a clinical problem in the dialysis population. The physiologic mechanisms leading to secondary hyperparathyroidism are multifactorial with renal phosphate retention, skeletal resistance to parathyroid hormone (PTH) action and impairment vitamin D metabolism being some of the known factors. Despite intensive medical management however inadequate control of parathyroid hyperplasia may necessitate surgical intervention. The goal of surgical therapy is to resect sufficient tissue to reverse the hyperparathyroidism without rendering the patient permanently hypoparathyroidism. We experienced a case of secondary hyperparathyroidism and reported its result of total parathyroidectomy, autogenous transplantation and cryopreservation of parathyroid gland.