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Sample records for neonatal severe hyperparathyroidism

  1. Neonatal, severe primary hyperparathyroidism: a 7-year clinical and radiological follow-up of one patient

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    Doria, Andrea S.; Daneman, Alan [Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario (Canada); Huang, Carol; Makitie, Outi; Kooh, Sang W.; Sochett, Etienne [Department of Endocrinology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8 (Canada); Thorner, Paul [Department of Laboratory Medicine, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario (Canada)

    2002-09-01

    Neonatal primary hyperparathyroidism is a rare entity characterized by marked hypercalcemia, diffuse parathyroid hyperplasia, and skeletal demineralization. It is often lethal unless total parathyroidectomy is performed. Long-term outcome of treated patients is poorly documented. We report the clinical and radiographic outcome of this disease in a 7-year-old boy who underwent a total parathyroidectomy and autotransplantation of a fragment of one parathyroid gland to his thigh in the neonatal period. This paper demonstrates the importance of prompt diagnosis and management in neonatal hyperparathyroidism and the role of various imaging modalities in its diagnosis and follow-up. (orig.)

  2. Cinacalcet Monotherapy in Neonatal Severe Hyperparathyroidism: A Case Study and Review

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    Gannon, Anthony W.; Monk, Heather M.

    2014-01-01

    Context: Neonatal severe hyperparathyroidism (NSHPT) is a severe form of familial hypocalciuric hypercalcemia characterized by severe hypercalcemia and skeletal demineralization. In most cases, NSHPT is due to biallelic loss-of-function mutations in the CASR gene encoding the calcium-sensing receptor (CaSR), but some patients have heterozygous mutations. Conventional treatment consists of iv saline, bisphosphonates, and parathyroidectomy. Objective: The aim of this project was to characterize the molecular basis for NSHPT in an affected newborn and to describe the response to monotherapy with cinacalcet. Methods: Clinical and biochemical features were monitored as cinacalcet therapy was initiated and maintained. Genomic DNA was obtained from the proband and parents. The CASR gene was amplified by PCR and sequenced directly. Results: The patient was a full-term male who developed hypotonia and respiratory failure soon after birth. He was found to have multiple fractures and diffuse bone demineralization, with a marked elevation in serum ionized calcium (1.99 mmol/L) and elevated serum levels of intact PTH (1154 pg/mL); serum 25-hydroxyvitamin D was low, and fractional excretion of calcium was reduced. The serum calcium level was not reduced by iv saline infusion. Based on an extensive family history of autosomal dominant hypercalcemia, a diagnosis of NSHPT was made, and cinacalcet therapy was initiated with a robust and durable effect. Molecular studies revealed a heterozygous R185Q missense mutation in the CASR in the patient and his father, whereas normal sequences for the CASR gene were present in the patient's mother. Conclusions: We describe the first use of cinacalcet as monotherapy for severe hypercalcemia in a newborn with NSHPT. The rapid and durable response to cinacalcet suggests that a trial of calcimimetic therapy should be considered early in the course of NSHPT. PMID:24203066

  3. Cinacalcet effect on severe hyperparathyroidism

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    Al Saran Khalid; Sabry Alaa; Yehia Ahmed; Molhem Azeb

    2010-01-01

    To determine the efficacy and safety of cinacalcet, a calcimimetic drug that suppress parathyroid hormone (PTH) production, we studied its effect on 20 patients (13 males) on maintenance hemodialysis (HD), 80% of them have persistent high PTH levels (i.e. more than 80 pmol/L), the remaining patients had PTH levels more than 60 pmol/L. Five of 20 (25%) patients dropped out from the study (2 because of severe GIT upset, one showed severe myalgia and arthralgia, one patient due to ...

  4. Cinacalcet effect on severe hyperparathyroidism

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    Al Saran Khalid

    2010-01-01

    Full Text Available To determine the efficacy and safety of cinacalcet, a calcimimetic drug that suppress parathyroid hormone (PTH production, we studied its effect on 20 patients (13 males on maintenance hemodialysis (HD, 80% of them have persistent high PTH levels (i.e. more than 80 pmol/L, the remaining patients had PTH levels more than 60 pmol/L. Five of 20 (25% patients dropped out from the study (2 because of severe GIT upset, one showed severe myalgia and arthralgia, one patient due to non compliance and one died at home due to cardiac arrest. The remaining 15 patients (10 males had a mean age of 40 ± 12.86 years and dialysis duration of 29.13 ± 18.27 months. The follow-up period on cinacalcet was 4 months with a single daily oral dose started with 30 mg/day and increased gradually according to the PTH levels. Nine (60% pa-tients were on concomitant active vitamin D during the study period with a mean dose of 7.33 ± 3.39 μg/week. There was a significant decrease in the serum PTH levels at the end the study compared to that at the start (46.4 ± 4.7 pmol/L versus 93.3 ± 25.6 pmol/L, respectively, P< 0.000, and the target PTH level (< 31.6 pmol/L was achieved in 54% of patients. No significant changes in serum Ca and phosphorous levels were observed. We conclude that cinacalcet is an effective therapy to suppress the serum PTH levels and allows favorable management of the serum calcium and phosphorus levels in HD patients. The drug was well tolerated; however, GIT discomfort is a significant side effect that may necessitate drug withdrawal in some patients.

  5. Late neonatal hypocalcemic tetany as a manifestation of unrecognized maternal primary hyperparathyroidism.

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    Çakır, Ufuk; Alan, Serdar; Erdeve, Ömer; Atasay, Begüm; Şıklar, Zeynep; Berberoğlu, Merih; Arslan, Saadet

    2013-01-01

    Maternal primary hyperparathyroidism causing hypercalcemia during pregnancy can suppress fetal and neonatal parathyroid hormone secretion. We report a newborn with transient hypoparathyroidism presented by hypocalcemic seizure and tetany on the 21st postnatal day in whom the final diagnosis was asymptomatic maternal primary hyperparathyroidism. Neonatal hypocalcemia usually occurs early in life in infants of maternal primary hyperparathyroidism, and although it is very rare, further investigation for unexplained late-onset hypocalcemia may reveal this diagnosis.

  6. [Severe macrocytic anaemia and secondary hyperparathyroidism in a vegan].

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    Førland, Elizabeth Siren Bjerga; Lindberg, Mats Jacob Hermansson

    2015-08-10

    Nutritional deficiency anaemia in vegans is common and usually due to lack of vitamin B12, as this vitamin is found almost exclusively in animal-based food products. In this case report we present a 39-year-old male vegan with severe macrocytic anaemia due to vitamin B12 deficiency as well as secondary hyperparathyroidism due to severe vitamin D deficiency. We want to emphasize the importance of a detailed nutritional history for patients with anaemia, and the need for vitamin B12 and vitamin D supplements for people who comply with a vegan diet.

  7. HYPERPARATHYROIDISM

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    Rooney, J. C.

    1949-01-01

    Because of the variable and vague clinical symptoms of the disease, diagnosis of hyperparathyroidism may be missed or delayed. Presenting symptoms and findings may be localized to the urological system or to the skeletal framework, with indications of abnormal blood calcium levels. In any case in which the patient's only complaints are malaise, lassitude, or progressive weakness, and in which routine laboratory findings are inconclusive, the blood calcium level should be determined. While not in itself diagnostic, a high level of calcium in the blood will lead to further investigation such as skeletal x-ray studies and blood phosphorus and serum alkaline phosphatase determinations. Vigilance postoperatively to forestall tetany is of great importance. Imagesp212-a PMID:18137218

  8. Severe neonatal hypercalcemia in 4-month-old, presented with respiratory distress and chest wall deformity

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    Akram Aljahdali

    2015-01-01

    Neonatal severe hyperparathyroidism (NSHPT) is a rare disease affecting calcium metabolism and results in severe life-treating hypocalcemia of the neonates. Diagnosis can be challenging due to variable and nonspecific symptomatology. We are reporting on a 4-month-old female infant presenting with respiratory distress and chest wall deformity. We are trying to highlight different surgical options for this rare disease and importance of close collaboration with the pediatric endocrinologist in ...

  9. Severe neonatal hypercalcemia in 4-month-old, presented with respiratory distress and chest wall deformity

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    Akram Aljahdali

    2015-01-01

    Full Text Available Neonatal severe hyperparathyroidism (NSHPT is a rare disease affecting calcium metabolism and results in severe life-treating hypocalcemia of the neonates. Diagnosis can be challenging due to variable and nonspecific symptomatology. We are reporting on a 4-month-old female infant presenting with respiratory distress and chest wall deformity. We are trying to highlight different surgical options for this rare disease and importance of close collaboration with the pediatric endocrinologist in the treatment plan for those patients.

  10. Prevalence of radiological findings among cases of severe secondary hyperparathyroidism

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    Paulo Gustavo Sampaio Lacativa

    Full Text Available CONTEXT AND OBJECTIVE: Patients with end stage renal disease (ESRD and secondary hyperparathyroidism (HPT2 are prone to develop heterotopic calcifications and severe bone disease. Determination of the sites most commonly affected would decrease costs and patients' exposure to X-ray radiation. The aim here was to determine which skeletal sites produce most radiographic findings, in order to evaluate hemodialysis patients with HPT2, and to describe the most prevalent radiographic findings. DESIGN AND SETTING: This study was cross-sectional, conducted in one center, the Hospital Universitário Clementino Fraga Filho (HUCFF, in Rio de Janeiro, Brazil. METHODS: Whole-body radiographs were obtained from 73 chronic hemodialysis patients with indications for parathyroidectomy due to severe HPT2. The regions studied were the skull, hands, wrists, clavicles, thoracic and lumbar column, long bones and pelvis. All the radiographs were analyzed by the same two radiologists, with great experience in bone disease interpretation. RESULTS: The most common abnormality was subperiosteal bone resorption, mostly at the phalanges and distal clavicles (94% of patients, each. "Rugger jersey spine" sign was found in 27%. Pathological fractures and deformities were seen in 27% and 33%, respectively. Calcifications were presented in 80%, mostly at the forearm fistula (42%, abdominal aorta and lower limb arteries (35% each. Brown tumors were present in 37% of the patients, mostly on the face and lower limbs (9% each. CONCLUSION: The greatest prevalence of bone findings were found on radiographs of the hands, wrists, lateral view of the thoracic and lumbar columns and femurs. The most prevalent findings were bone resorption and ectopic calcifications.

  11. Cinacalcet reduces plasma intact parathyroid hormone, serum phosphate and calcium levels in patients with secondary hyperparathyroidism irrespective of its severity.

    LENUS (Irish Health Repository)

    2011-09-01

    To evaluate the relationship between the severity of secondary hyperparathyroidism (SHPT) - defined in terms of baseline plasma intact parathyroid hormone (iPTH) level - and the magnitude of response to cinacalcet.

  12. Persistent Primary Hyperparathyroidism, Severe Vitamin D Deficiency, and Multiple Pathological Fractures

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    Victoria Mendoza-Zubieta

    2016-01-01

    Full Text Available Persistent primary hyperparathyroidism (PHPT refers to the sustained hypercalcemia state detected within the first six months following parathyroidectomy. When it coexists with severe vitamin D deficiency, the effects on bone can be devastating. We report the case of a 56-year-old woman who was sent to this center because of persistent hyperparathyroidism. Her disease had over 3 years of evolution with nephrolithiasis and hip fracture. Parathyroidectomy was performed in her local unit; however, she continued with hypercalcemia, bone pain, and pathological fractures. On admission, the patient was bedridden with multiple deformations by fractures in thoracic and pelvic members. Blood pressure was 100/80, heart rate was 86 per minute, and body mass index was 19 kg/m2. Calcium was 14 mg/dL, parathormone 1648 pg/mL, phosphorus 2.3 mg/dL, creatinine 2.4 mg/dL, urea 59 mg/dL, alkaline phosphatase 1580 U/L, and vitamin D 4 ng/mL. She received parenteral treatment of hypercalcemia and replenishment of vitamin D. The second surgical exploration was radioguided by gamma probe. A retroesophageal adenoma of 4 cm was resected. Conclusion. Persistent hyperparathyroidism with severe vitamin D deficiency can cause catastrophic skeletal bone softening and fractures.

  13. Persistent Primary Hyperparathyroidism, Severe Vitamin D Deficiency, and Multiple Pathological Fractures

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    Carvallo-Venegas, Mauricio; Vargas-Castilla, Jorge Alberto; Balcázar-Hernández, Lourdes Josefina; Gregor-Gooch, Julián Malcolm Mac

    2016-01-01

    Persistent primary hyperparathyroidism (PHPT) refers to the sustained hypercalcemia state detected within the first six months following parathyroidectomy. When it coexists with severe vitamin D deficiency, the effects on bone can be devastating. We report the case of a 56-year-old woman who was sent to this center because of persistent hyperparathyroidism. Her disease had over 3 years of evolution with nephrolithiasis and hip fracture. Parathyroidectomy was performed in her local unit; however, she continued with hypercalcemia, bone pain, and pathological fractures. On admission, the patient was bedridden with multiple deformations by fractures in thoracic and pelvic members. Blood pressure was 100/80, heart rate was 86 per minute, and body mass index was 19 kg/m2. Calcium was 14 mg/dL, parathormone 1648 pg/mL, phosphorus 2.3 mg/dL, creatinine 2.4 mg/dL, urea 59 mg/dL, alkaline phosphatase 1580 U/L, and vitamin D 4 ng/mL. She received parenteral treatment of hypercalcemia and replenishment of vitamin D. The second surgical exploration was radioguided by gamma probe. A retroesophageal adenoma of 4 cm was resected. Conclusion. Persistent hyperparathyroidism with severe vitamin D deficiency can cause catastrophic skeletal bone softening and fractures. PMID:27525132

  14. Primary hyperparathyroidism

    OpenAIRE

    Madkhali, Tarıq; Alhefdhi, Amal; Chen, Herbert; Elfenbein, Dawn

    2016-01-01

    Primary hyperparathyroidism is a common endocrine disorder caused by overactivation of parathyroid glands resulting in excessive release of parathyroid hormone. The resultant hypercalcemia leads to a myriad of symptoms. Primary hyperparathyroidism may increase a patient’s morbidity and even mortality if left untreated. During the last few decades, disease presentation has shifted from the classic presentation of severe bone and kidney manifestations to most patients now being diagnosed on rou...

  15. [Multiple brown tumors in a female hemodialyzed patient with severe secondary hyperparathyroidism].

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    Peces, R; Gil, F; González, F; Ablanedo, P

    2002-01-01

    Skeletal brown tumours are relatively uncommon, and brown tumours that involve multiple bones are considered very rare. We describe a 29-year-old woman with chronic renal failure (CRF) who had undergone hemodialysis for 21 years and developed multiple brown tumours associated with severe secondary hyperparathyroidism. Computed tomography (CT) revealed multiple brown tumours involving scapula, ribs, spine and sacroiliac bone. Microscopic analysis of the brown tumour showed dense infiltration of the marrow space by reactive fibroblastic tissue with irregularly distributed multinucleated osteoclastic giants cells and marked increase in hematopoietic elements.

  16. Pseudoarthrosis and fracture: interaction between severe vitamin D deficiency and primary hyperparathyroidism.

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    Rastogi, Ashu; Bhadada, Sanjay Kumar; Bhansali, Anil

    2013-11-01

    A young woman with severe vitamin D deficiency presented with proximal muscle weakness, fragility fracture and pseudoarthrosis. On evaluation, she was found to have hypercalcaemia, a single parathyroid adenoma and an undetectable 25-hydroxyvitamin D level. She received parenteral cholecalciferol and subsequently underwent curative parathyroidectomy. Postoperatively, she had hungry bone syndrome, which she gradually recovered from with calcium and calcitriol replacement. Notably, her calcium levels were in the lower limit of normal range and associated with elevated alkaline phosphatase levels at postoperative Day 14. Follow-up for the next four years showed that the patient had remarkable symptomatic and radiological improvements. In this report, we discuss the pathophysiological interactions between vitamin D deficiency and associated primary hyperparathyroidism.

  17. A novel dose regimen of cinacalcet in the treatment of severe hyperparathyroidism in hemodialysis patients

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    Nabieh Al-Hilali

    2011-01-01

    Full Text Available During the recent years, cinacalcet has markedly improved the management of hyperparathyroidism in patients on hemodialysis. However, to the best of our knowledge, there are no specific studies addressing the dose regimen of cinacalcet. The aim of the study was to evaluate the efficacy of cinacalcet on the achievement of targets in the treatment of hyperparathyroidism in two different dosage schedules. Twenty-seven adult patients who were on hemodialysis for more than four months and with severe secondary hyperparathyroidism (intact parathyroid hormone (iPTH >88 pmol/L resistant to conventional treatment were included in this prospective study. We used the targets of K/DOQI-clinical guidelines as optimal target of iPTH, calcium and phosphate. Group 1 received a single daily administration of 30 mg of cinacalcet along with the main meal as the starting dose, and the dose was titrated thereafter monthly. Group 2 received cinacalcet with the main meal twice weekly starting with a dose of 90 mg on the first day of the week and 120 mg at midweek and titrated thereafter monthly. The levels of iPTH decreased significantly (P = 0.0001 from 124.00 ± 44.77 pmol/L to 37.78 ± 12.49 pmol/L and from 109.61 ± 53.13 pmol/L to 33.93 ± 12.03 pmol/L after 12 weeks in groups 1 and 2, respectively. After 12 weeks, alkaline phosphatase declined significantly (P = 0.0001 from 143.42 ± 75.20 IU/L to 87.42 ± 14.46 IU/L in group 1 (P = 0.013, and from 148.00 ± 108.49 IU/L to 101.61 ± 46.62 IU/L in group 2 (P = 0.05. There were no significant differences between the reductions of iPTH, calcium phosphate product and alkaline phosphatase levels in both the groups in the vertical comparison at the end of the study. There was no noteworthy difference in side effects between both the groups. Our results indicate that cinacalcet twice weekly is reasonably safe and effective in suppressing high PTH levels in hemodialysis patients, with fewer side effects.

  18. Severe Neonatal Hyperbilirubinemia in the Netherlands

    NARCIS (Netherlands)

    Gotink, Mark J.; Benders, Manon J.; Lavrijsen, Selma W.; Pereira, Rob Rodrigues; Hulzebos, Christian V.; Dijk, Peter H.

    2013-01-01

    Background: The occurrence of severe neonatal hyperbilirubinemia (SH) is partly attributed to nonhospitalized perinatal care. The Netherlands have a high frequency of home births and nonhospitalized perinatal care, and the incidence of SH is unknown. Objective: To assess the effects of home births a

  19. Efficacy of microwave ablation for severe secondary hyperparathyroidism in subjects undergoing hemodialysis.

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    Diao, Zongli; Wang, Liyan; Li, Dishan; Liu, Wenhu

    2017-11-01

    Severe secondary hyperparathyroidism (SHPT) is a serious problem in patients undergoing hemodialysis. The efficacy and safety of microwave ablation (MWA), a minimally invasive treatment, for severe SHPT are as yet unclear. To clarify the role of MWA, we administered it to patients with severe SHPT and assessed its efficacy and safety. This was a prospective, single-center, single-arm, clinical trial. We enrolled patients with severe SHPT attending our hemodialysis center who met the inclusion and exclusion criteria. We then assessed primary outcome measures (serum concentrations of intact parathyroid hormone) and secondary outcome measures (serum concentrations of calcium and phosphorus). Twenty-six patients were enrolled in this study, 10 of whom (38.46%) were responsive to MWA and 16 (61.54%) of whom were not. The main complication was hypocalcemia (10 cases, 38.46%), which had occurred in all cases by one week after administration of MWA. Responding patients with hypocalcemia all achieved normal serum calcium concentrations within seven months and non-responding patients within three months. There were no changes in serum phosphorus concentrations after MWA in either responders or non-responders. Microwave ablation is relatively ineffective in patients with severe SHPT undergoing maintaining hemodialysis and should not be the initial therapy in such cases.

  20. A novel CaSR mutation presenting as a severe case of neonatal familial hypocalciuric hypercalcemia

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    Tonyushkina Ksenia N

    2012-05-01

    Full Text Available Abstract Background Familial Hypocalciuric Hypercalcemia (FHH is a generally benign disorder caused by heterozygous inactivating mutations in the Calcium-Sensing Receptor (CaSR gene resulting in altered calcium metabolism. Objective We report a case of unusually severe neonatal FHH due to a novel CaSR gene mutation that presented with perinatal fractures and moderate hypercalcemia. Case overview A female infant was admitted at 2 weeks of age for suspected non-accidental trauma (NAT. Laboratory testing revealed hypercalcemia (3.08 mmol/L, elevated iPTH (20.4 pmol/L and low urinary calcium clearance (0.0004. Radiographs demonstrated multiple healing metaphyseal and rib fractures and bilateral femoral bowing. The femoral deformity and stage of healing were consistent with prenatal injuries rather than non-accidental trauma (NAT. Treatment was initiated with cholecalciferol, 400 IU/day, and by 6 weeks of age, iPTH levels had decreased into the high-normal range. Follow up radiographs demonstrated marked improvement of bone lesions by 3 months. A CaSR gene mutation study showed heterozygosity for a T>C nucleotide substitution at c.1664 in exon 6, resulting in amino acid change I555T in the extracellular domain consistent with a missense mutation. Her mother does not carry the mutation and the father is unknown. At 18 months of age, the child continues to have relative hyperparathyroidism and moderate hypercalcemia but is otherwise normal. Conclusion This neonate with intrauterine fractures and demineralization, moderate hypercalcemia and hyperparathyroidism was found to have a novel inactivating missense mutation of the CaSR not detected in her mother. Resolution of bone lesions and reduction of hyperparathyroidism was likely attributable to the natural evolution of the disorder in infancy as well as the mitigating effect of cholecalciferol treatment.

  1. Anaesthetic Implications in Primary Hyperparathyroidism with Severe Hypercalcaemia; a Case Report

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    Pranav Bansal

    2013-07-01

    Full Text Available Primary hyperparathyroidism is a rare endocrinal disorder of excess production of parathormone. A wide array of presenting symptoms may occur from parathormone induced hypercalcaemia leading to nephrolithiasis, osteoporosis, muscle weakness and cardiac arrhythmias. We present a case of young female who presented with non union of an operated fracture femur and generalized bony pains and frequent complaints of vomiting, polyuria and polydipsia. She was diagnosed to have primary hyperparathyroidism with hypercalcaemia and underwent parathyroidectomy. The potential perioperative problems and anaesthetic concerns require a focused management and are discussed.

  2. Long-term treatment with cinacalcet and conventional therapy reduces parathyroid hyperplasia in severe secondary hyperparathyroidism

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    Meola, Mario; Petrucci, Ilaria; Barsotti, Giuliano

    2009-01-01

    Background. The effect of cinacalcet on the structural pattern of hyperplastic parathyroid glands was evaluated, using high-resolution colour Doppler (CD) sonography, in haemodialysis patients with severe, inadequately controlled, secondary hyperparathyroidism (sHPT). Methods. Nine patients (6 males, 3 females; mean age ± SD, 55.5 ± 12.6 years) received cinacalcet, with adaptation of existing concomitant therapies. Biochemical parameters and the morphology and vascular pattern of hyperplastic parathyroid glands were measured at baseline and every 6 months thereafter, for a follow-up period of 24–30 months. Results. At baseline, 28 hyperplastic glands were identified. Cinacalcet led to a reduction in glandular volume during the course of the study: 68% in glands with a baseline volume <500 mm3 and 54% in glands with a baseline volume ≥500 mm3. The mean volume ± SD of glands <500 mm3 changed significantly from the baseline (233 ± 115 mm3) to the end of follow-up (102 ± 132 mm3, P = 0.007). Levels of mean serum phosphorus, calcium and calcium–phosphorus product decreased, but not significantly, whereas there were significant decreases in mean parathyroid hormone ± SD levels (1196 ± 381 pg/ml versus 256 ± 160 pg/ml; P < 0.0001) and alkaline phosphatase ± SD levels (428 ± 294 versus 223 ± 88 IU/l; P = 0.04), accompanied by an improvement in a subjective clinical score. Conclusions. Cinacalcet, in combination with conventional treatments, led to an improvement in biochemical and clinical parameters of sHPT and reduced glandular volume in patients with severe sHPT. Volume reduction was more evident in smaller glands. Longer term, larger, randomized clinical trials are needed to confirm these preliminary findings and to further define a more systematic approach in the treatment of sHPT. PMID:19181759

  3. Cinacalcet HCl Reduces Hypercalcemia in Primary Hyperparathyroidism across a Wide Spectrum of Disease Severity

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    Peacock, Munro; Bilezikian, J. P.; Bolognese, M. A.; Borofsky, Michael; Scumpia, Simona; Sterling, L. R.; Cheng, Sunfa; Shoback, Dolores

    2011-01-01

    Context: Primary hyperparathyroidism (PHPT) is characterized by elevated serum calcium (Ca) and increased PTH concentrations. Objective: The objective of the investigation was to establish the efficacy of cinacalcet in reducing serum Ca in patients with PHPT across a wide spectrum of disease severity. Design and Setting: The study was a pooled analysis of data from three multicenter clinical trials of cinacalcet in PHPT. Patients : Patients were grouped into three disease categories for analysis based on the following: 1) history of failed parathyroidectomy (n = 29); 2) meeting one or more criteria for parathyroidectomy but without prior surgery (n = 37); and 3) mild asymptomatic PHPT without meeting criteria for either above category (n = 15). Intervention: The intervention in this study was treatment with cinacalcet for up to 4.5 yr. Outcomes: Measurements in the study included serum Ca, PTH, phosphate, and bone-specific alkaline phosphatase, and areal bone mineral density (aBMD). Vital signs, safety biochemical and hematological indices, and adverse events were monitored throughout the study period. Results: The extent of cinacalcet-induced serum Ca reduction, proportion of patients achieving normal serum Ca (≤10.3 mg/dl), reduction in serum PTH, and increase in serum phosphate were similar across all three categories. Except for decreased aBMD at the total femur indicated for parathyroidectomy group at 1 yr, no significant changes in aBMD occurred. The efficacy of cinacalcet was maintained for up to 4.5 yr of follow-up. AEs were mild and similar across the three categories. Conclusions: Cinacalcet is equally effective in the medical management of PHPT patients across a broad spectrum of disease severity, and overall cinacalcet is well tolerated. PMID:20943783

  4. Severe Combined Immunodeficiency (SCID) Presenting with Neonatal Aplastic Anemia

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    Scott, Angela; Glover, Jason; Skoda-Smith, Suzanne; Torgerson, Troy; Xu, Min; Burroughs, Lauri; Woolfrey, Ann; Fleming, Mark; Shimamura, Akiko

    2015-01-01

    Aplastic anemia in the neonate is rare. We report a case of severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia. This report highlights the importance of considering SCID early in the evaluation of neonatal aplastic anemia prior to the development of infectious complications. PMID:26011426

  5. Severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia.

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    Scott, Angela; Glover, Jason; Skoda-Smith, Suzanne; Torgerson, Troy R; Xu, Min; Burroughs, Lauri M; Woolfrey, Ann E; Fleming, Mark D; Shimamura, Akiko

    2015-11-01

    Aplastic anemia in the neonate is rare. We report a case of severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia. This report highlights the importance of considering SCID early in the evaluation of neonatal aplastic anemia prior to the development of infectious complications. © 2015 Wiley Periodicals, Inc.

  6. Severe neonatal parechovirus infection and similarity with enterovirus infection

    NARCIS (Netherlands)

    Verboon-Maciolek, Malgorzata A.; Krediet, Tannette G.; Gerards, Leo J.; de Vries, Linda S.; Groenendaal, Floris; van Loon, Anton M.

    Background: Enteroviruses (EV) are an important cause of neonatal disease including hepatitis, meningoencephalitis, and myocarditis that can lead to death or severe long-term sequelae. Less is known about severe neonatal infection caused by the parechoviruses (PeV) of which type 1 (PeV1) and type 2

  7. Tolerance and efficacy of a low dose of the calcimimetic agent cinacalcet in controlling moderate to severe secondary hyperparathyroidism in hemodialysis patients

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    Salah O Bashir

    2015-01-01

    Full Text Available Secondary hyperparathyroidism is almost a constant feature in chronic kidney disease (CKD patients maintained on hemodialysis (HD. Calcimimetic agents appear to offer an alternative to surgery in controlling secondary hyperparathyroidism in these patients. Recent studies provide conflicting data on the benefits, efficacy and tolerance of cinacalcet as first-line therapy for the treatment of secondary hyperparathyroidism in CKD. This study was designed to investigate the efficacy and tolerance of a low dose of the calcimimetic agent cinacalcet in patients on long-term HD having moderate to severe secondary hyperparathyroidism. Twentyfive adult male patients on HD for more than three years were included in the study. All had moderate to severe secondary hyperparathyroidism with serum intact parathyroid hormone (iPTH >50 pmol/L, resistant to conventional treatment. We used the targets of Chronic Kidney Disease: Outcomes Quality Initiative (K/DOQI clinical guidelines as optimal target of serum iPTH, calcium and phosphate. Patients were administered cinacalcet as a single oral daily dose of 30 mg and were followed-up for six months. Cinacalcet treatment for six months resulted in a significant reduction in the serum phosphate and iPTH levels while the serum calcium levels remained unchanged. Thirty-six percent of the patients attained the recommended serum iPTH levels, 40% achieved significant reduction of the serum iPTH levels and 24% showed no favorable response. Only one patient dropped out because of severe gastrointestinal symptoms. Our results suggest that treatment of CKD patients, having moderate to severe secondary hyperparathyroidism, with low-dose cinacalcet is effective and well tolerated.

  8. Primary hyperparathyroidism.

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    Muñoz-Torres, Manuel; García-Martín, Antonia

    2017-10-06

    Primary hyperparathyroidism (PHPT) is a common endocrinological process, characterized by chronic elevation of serum concentrations of calcium and parathyroid hormone (PTH). Many years ago, the most frequent forms of clinical presentation were symptomatic renal or skeletal disease with moderate or severe hypercalcemia; however, currently, most patients have few symptoms and mild hypercalcemia. A new form of presentation called normocalcemic PHPT has also been described but clinical consequences are not well established. The biochemical profile of PHPT is characterized by hypercalcemia and high or inappropriately normal PTH concentrations. Parathyroidectomy is the only definitive cure. Medical treatment with the calcimimetic cinacalcet has been shown to normalize calcemia in a high percentage of cases. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  9. Treatment of CRF Patients with Severe Secondary Hyperparathyroidism by Parathyroidectomy Combined with Parathyroid Tissue Transplantation in Forearm(Report of 10 Cases)

    Institute of Scientific and Technical Information of China (English)

    WuHF; WangXY

    2002-01-01

    Objective To investigate the efficacy of parathyroidectomy combined with parathyroid tissue transplantation in forearm in treatment of CRF patients with severe secondary hyperparathyroidism(SHP).Methods 10 cases of CRF with SHP were treated with parathyroidectomy combined with parathyroid tissue transplantation in forearm from 1995 to 2001.The diagnosis,operation indication and peri-operation management of ten patients were discussed.Results The hypertrophy parathyroids were totally resected in 10 CRF cases with SHP.All grafts were alive after transplantation.Symptoms of hyperparathyroidism disappeared and the various laboratory test turned to normal after operation.Conclusion That surgical manipulation is safe and effective in those CRF patients with severe SHP who failed in medical therapy.

  10. Screening for severe combined immunodeficiency in neonates

    OpenAIRE

    Kelly BT; Tam JS; Verbsky JW; Routes JM

    2013-01-01

    Brian T Kelly,1 Jonathan S Tam,1 James W Verbsky,1,2 John M Routes1,2 1Department of Pediatrics, 2Department of Microbiology and Molecular Genetics, Medical College of Wisconsin, Milwaukee, WI, USA Abstract: Severe combined immunodeficiency (SCID) is a rare disease that severely affects the cellular and humoral immune systems. Patients with SCID present with recurrent or severe infections and often with chronic diarrhea and failure to thrive. The disease is uniformly fatal, making early diag...

  11. Screening for severe combined immunodeficiency in neonates

    Science.gov (United States)

    Kelly, Brian T; Tam, Jonathan S; Verbsky, James W; Routes, John M

    2013-01-01

    Severe combined immunodeficiency (SCID) is a rare disease that severely affects the cellular and humoral immune systems. Patients with SCID present with recurrent or severe infections and often with chronic diarrhea and failure to thrive. The disease is uniformly fatal, making early diagnosis essential. Definitive treatment is hematopoietic stem cell transplantation, with best outcomes prior to 3.5 months of age. Newborn screening for SCID using the T-cell receptor excision circle assay has revolutionized early identification of infants with SCID or severe T-cell lymphopenia. PMID:24068875

  12. Screening for severe combined immunodeficiency in neonates

    Directory of Open Access Journals (Sweden)

    Kelly BT

    2013-09-01

    Full Text Available Brian T Kelly,1 Jonathan S Tam,1 James W Verbsky,1,2 John M Routes1,2 1Department of Pediatrics, 2Department of Microbiology and Molecular Genetics, Medical College of Wisconsin, Milwaukee, WI, USA Abstract: Severe combined immunodeficiency (SCID is a rare disease that severely affects the cellular and humoral immune systems. Patients with SCID present with recurrent or severe infections and often with chronic diarrhea and failure to thrive. The disease is uniformly fatal, making early diagnosis essential. Definitive treatment is hematopoietic stem cell transplantation, with best outcomes prior to 3.5 months of age. Newborn screening for SCID using the T-cell receptor excision circle assay has revolutionized early identification of infants with SCID or severe T-cell lymphopenia. Keywords: severe combined immunodeficiency, T-cell receptor excision circle, newborn screening, primary immunodeficiency

  13. Double Volume Exchange Transfusion in Severe Neonatal Sepsis.

    Science.gov (United States)

    Aradhya, Abhishek Somasekhara; Sundaram, Venkataseshan; Kumar, Praveen; Ganapathy, Suja Mariam; Jain, Ashish; Rawat, Amit

    2016-02-01

    To study the efficacy and safety of double volume exchange transfusion (DVET) in neonates > 1000 g birth weight with severe sepsis. Eighty-three neonates weighing >1000 g with severe sepsis were randomly assigned to DVET or standard therapy (ST) group. Primary outcome was mortality by 14 d from enrollment. A 21 % reduction in mortality, albeit non-significant, by 14 d from enrollment was observed in DVET group in comparison to ST group [RR: 0.79 (95 % C.I 0.45-1.3); p 0.4]. A similar trend in mortality reduction was observed with early mortality and mortality by discharge in DVET group. No difference was observed in normalization of dysfunctional organs by 14 d. Cardiovascular and hematological system benefitted the most, followed by renal dysfunction with DVET. A significant improvement in post DVET IgG, IgA, IgM, C3 and base deficit was observed. No serious adverse effects occurred following DVET. In neonates >1000 g with severe sepsis, DVET was associated with a trend towards decrease in mortality by 14 d from enrollment. A significant improvement in immunoglobulin and complement C3 levels and acid base status were observed following DVET. DVET is a safe procedure in severely sick and septic neonates.

  14. Neonatal Screening for Severe Combined Immunodeficiency (SCID)

    Science.gov (United States)

    Puck, Jennifer M.

    2012-01-01

    Purpose of review Population-based newborn screening for severe combined immunodeficiency (SCID) and related disorders has been instituted in five states, with several more planning to add this testing to their newborn screening panels. This review summarizes the rationale, development, and implementation of SCID screening programs to date and highlights current and future challenges. Recent findings Early results of T-cell receptor excision circle (TREC) testing newborns in pilot states indicate that this addition to the newborn screening panel can be successfully integrated into state public health programs. The TREC test has clinical validity and TRECs, as predicted, are an excellent biomarker of poor T-cell lymphocyte production in the thymus or increased lymphocyte loss resulting in T-cell lymphopenia. A variety of cases with typical SCID genotypes and other conditions have been detected in a timely manner and referred for appropriate early treatment. Summary Early detection of primary immunodeficiency is recognized as important for avoiding infectious complications that compromise outcomes. Routine screening of all newborns with the TREC test, implemented as part of an integrated public health program, can achieve pre-symptomatic diagnosis of SCID and other disorders with T-cell lymphopenia, allowing prompt and effective treatment and leading to a better understanding of the spectrum of these disorders and how to manage them. PMID:22001765

  15. Severe neonatal epileptic encephalopathy and KCNQ2 mutation: neuropathological substrate?

    Directory of Open Access Journals (Sweden)

    Charlotte eDalen Meurs-Van Der Schoor

    2014-12-01

    Full Text Available Background:Neonatal convulsions are clinical manifestations in a heterogeneous group of disorders with different etiology and outcome. They are attributed to several genetic causes. Methods:We describe a patient with intractable neonatal seizures who died from respiratory compromise during a status epilepticus. Results:This case report provides EEG, MRI, genetic analysis and neuropathological data. Genetic analysis revealed a de novo heterozygous missense mutation in the KCNQ2 gene, which encodes a subunit of a voltage-gated potassium channel. KCNQ2 gene mutation is associated with intractable neonatal seizures. EEG, MRI data as well as mutation analysis have been described in other KCNQ2 cases. Postmortem neuropathologic investigation revealed mild malformation of cortical development with increased heterotopic neurons in the deep white matter compared to an age-matched control subject. The new finding of this study is the combination of a KCNQ2 mutation and the cortical abnormalities. Conclusions:KCNQ2 mutations should be considered in neonates with refractory epilepsy of unknown cause. The mild cortical malformation is an important new finding, though it remains unknown whether these cortical abnormalities are due to the KCNQ2 mutation or are secondary to the refractory seizures.

  16. Primary hyperparathyroidism and nephrolithiasis.

    Science.gov (United States)

    Vestergaard, Peter

    2015-05-01

    Calcifications in the kidneys may occur in the parenchyma (nephrocalcinosis), pelvis renis (nephrolithiasis) or ureters (ureterolithiasis). Several factors may protect against stone formation or promote precipitation of stones. Most stones contain calcium, and the hypercalciuria seen in primary hyperparathyroidism is a contributing factor to stone formation in the kidneys and urinary tract. In early case series, renal stone formation was frequent, whereas the proportion of patients with symptomatic renal stones has declined in recent years. However, a substantial proportion of patients presents with asymptomatic nephrocalcinosis or nephrolithiasis. Before diagnosis and treatment of primary hyperparathyroidism, renal stone events are more frequent than in the general population. However, even after surgical cure, an increased rate of renal stone events may be seen. This may to some extent be the result of stones or calcifications already present at the time of diagnosis or sequelae to prior stones such as infections or ureter strictures.

  17. Severe Blue Rubber Bleb Nevus Syndrome in a Neonate

    DEFF Research Database (Denmark)

    Hansen, L; Wewer, V; Pedersen, S

    2008-01-01

    bleeding during the first 3 years of life. Afterwards repeated endoscopic electrocoagulations were performed over a period of one year resulting in a termination of bleeding episodes. At ten years of age the patient developed spastic diplegia with slight walking disabilities, coordination and fine motor...... problems. The case is unique because 1) it is the first neonatal case with BRBNS and severe gastrointestinal bleeding; 2) the patient was successfully treated by endoscopic electrocoagulation; and 3) the long-term follow-up. The use of electrocoagulation appears to have been effective and ablation...

  18. Severe Blue Rubber Bleb Nevus Syndrome in a Neonate

    DEFF Research Database (Denmark)

    Hansen, L; Wewer, V; Pedersen, S;

    2008-01-01

    bleeding during the first 3 years of life. Afterwards repeated endoscopic electrocoagulations were performed over a period of one year resulting in a termination of bleeding episodes. At ten years of age the patient developed spastic diplegia with slight walking disabilities, coordination and fine motor...... problems. The case is unique because 1) it is the first neonatal case with BRBNS and severe gastrointestinal bleeding; 2) the patient was successfully treated by endoscopic electrocoagulation; and 3) the long-term follow-up. The use of electrocoagulation appears to have been effective and ablation...

  19. Primary hyperparathyroidism in children and adolescents

    Directory of Open Access Journals (Sweden)

    Jeffrey Roizen

    2012-09-01

    Full Text Available Primary hyperparathyroidism (PHPT is a common endocrine disorder in adults in whom the typical presentation is incidentally discovered as asymptomatic hypercalcemia. PHPT is much less common in children and adolescents, but has greater morbidity in this age group, as most young patients with PHPT will have symptomatic hypercalcemia or complications such as kidney stones, abdominal pain, and skeletal fragility. An important feature of PHPT in younger patients is the relatively high prevalence of germline inactivating mutations of the CASR gene, which encodes the calcium-sensing receptor. Biallelic CASR mutations cause neonatal severe hyperparathyroidism, a life-threatening condition that presents within days of life with marked hypercalcemia, respiratory distress, failure to thrive, and skeletal demineralization. By contrast, more common heterozygous CASR mutations are generally associated with a benign variant of PHPT termed familial hypocalciuric hypercalcemia. Appropriate management of PHPT in children and adolescents requires distinction between familial hypocalciuric hypercalcemia, which generally requires no specific treatment, and other forms of PHPT that are best treated by parathyroidectomy.

  20. Turner syndrome with primary hyperparathyroidism

    Science.gov (United States)

    Park, Jungmee; Kim, Yoo-Mi; Choi, Jin-Ho; Lee, Beom Hee; Yoon, Jong Ho; Jeong, Woon-Young

    2013-01-01

    Turner syndrome has multiple comorbidities such as osteoporosis, obesity, diabetes, hypothyroidism, and hypertension. As they are treatable conditions in Turner syndrome, early recognition and proper treatment should be needed. We report on a 23-year-old woman with Turner syndrome who presented with severe osteoporosis and hypercalcemia. Laboratory tests showed elevated levels of serum calcium and parathyroid hormone. Dual-energy X-ray absorptiometry showed severe osteopo-rosis (z score, -3.5). Ultrasound and 99mTc scintigraphy of parathyroid glands showed an adenoma in the right inferior gland. She was diagnosed with primary hyperparathyroidism due to an adenoma of the parathyroid gland. After excision of the adenoma, the patient's serum calcium and parathyroid hormone levels returned to normal. Although only a few cases of Turners syndrome with primary hyperparathyroidism have been reported, hyperparathyroidism should be considered in cases of Turner syndrome with severe osteoporosis and hypercalcemia. PMID:24904858

  1. Self-reported symptoms in patients on hemodialysis with moderate to severe secondary hyperparathyroidism receiving combined therapy with cinacalcet and low-dose vitamin D sterols.

    Science.gov (United States)

    Chertow, Glenn M; Lu, Z John; Xu, Xiao; Knight, Tyler G; Goodman, William G; Bushinsky, David A; Block, Geoffrey A

    2012-04-01

    Patients with secondary hyperparathyroidism experience a variety of clinical symptoms which may adversely affect physical and mental function. As part of a multicenter, open-label clinical trial, subjects completed a questionnaire that included the Medical Outcomes Study Short Form-36 and 14 kidney disease-related symptoms at multiple time points during the study. Out of the 567 subjects who received at least one dose of cinacalcet, 528 to 535 (93.8-94.4%) completed all or portions of the questionnaire at baseline. The median bioactive parathyroid hormone (PTH) was 294 pg/mL (10%, 90% range, 172-655 pg/mL). Following treatment with cinacalcet and low-dose vitamin D sterols, subjects reported significant improvement in the frequency of pain in muscles, joints and bones, stiff joints, dry skin, itchy skin, excessive thirst, and trouble with memory. At end of the efficacy assessment phase (Weeks 16 to 22), the magnitude of improvement was the greatest in joint pain, bone pain, dry skin, and excessive thirst (>5 on a 0-100 scale; P clinically or statistically significant changes in any of the Short Form-36 subscales or in the physical or mental health composite scores. Among patients on hemodialysis with moderate to severe secondary hyperparathyroidism, treatment with cinacalcet and low-dose vitamin D sterols results in significant improvement in pain in the muscles, joints and bones, joint stiffness, dry and itchy skin, excessive thirst, and trouble with memory.

  2. Neonatal side effects of maternal labetalol treatment in severe preeclampsia.

    Science.gov (United States)

    Heida, Karst Y; Zeeman, Gerda G; Van Veen, Teelkien R; Hulzebos, Christian V

    2012-07-01

    Labetalol is often used in severe preeclampsia (PE). Hypotension, bradycardia and hypoglycemia are feared neonatal side effects, but may also occur in (preterm) infants regardless of labetalol exposure. We analyzed the possible association between intrauterine labetalol exposure and such side effects. From 1 January 2003 through 31 March 2008, all infants from mothers suffering severe PE admitted to one tertiary care center were included. Severe PE was defined according to the International Society for the Study of Hypertension in Pregnancy (ISSHP) criteria. Infants exposed to labetalol in utero (labetalol infants) were compared with infants, who were not exposed to labetalol (controls). Neonatal records were reviewed for hypotension (RRheartrate<100/min) and hypoglycaemia (glucose<2.7 mmol/L) in the first 48 postnatal hours. Of 109 infants, 55 had been exposed to labetalol, whereas 54 were not (controls). Gestational age at delivery and birthweight were similar in both groups (31.8 vs. 32.8 weeks (p=0.06) and 1510 vs. 1639 grams (p=0.25), respectively for the labetalol vs. control group). Hypotension occurred significantly more in conjunction with labetalol exposure (16, (29.1%) vs. 4 (7.4%); p=0.003), irrespective of the route of administration. Patent ductus arteriosus (PDA) was present in 9 (56%) of hypotensive labetalol infants compared to 1 (24%) infant in the hypotensive control group (NS). In a multivariate regression model, labetalol exposure, the need for intubation and PDA appeared independently associated with hypotension (P<0.001). Hypoglycemia occurred in 26 (47.3%) of labetalol infants and in 23 (42.6%) of control infants (p=0.62). Bradycardia occurred in 4 (7.3%) of labetalol infants and in 1 (1.9%) of control infants (p=0.18). Hypoglycemia was more common in premature infants (n=45 (48,9%) vs. n=4 (23.5%), p=0.05) in both labetalol and control infants. Hypotension is more common after maternal labetalol exposure, regardless of the dosage and route

  3. Neonates presenting with severe complications of frenotomy: a case series

    Directory of Open Access Journals (Sweden)

    Opara Peace I

    2012-03-01

    Full Text Available Abstract Introduction Tongue-tie or ankyloglossia is an anatomic variation in which the lingual frenulum is thick, short or tight. It may be asymptomatic, or present with complications like breast feeding difficulties or speech, dental and cosmetic problems. The treatment of this condition, where indicated, is frenotomy. This procedure usually has few or no complications. However, when it is done by untrained personnel, it may lead to life-threatening complications. This paper highlights complications that could arise from improper treatment of ankyloglossia. Case presentation Case 1 was a one-day-old male neonate, a Nigerian of Igbo ethnicity, who was admitted with bleeding from the mouth and passage of dark stools after clipping of the frenulum by a traditional birth attendant. He was severely pale and in hypovolemic shock, with a severed frenulum which was bleeding actively. His packed cell volume was 15%. He was resuscitated with intravenous fluids and a blood transfusion. The bleeding was controlled using an adrenaline pack. He also received antibiotics. He was discharged five days later. Case 2 was a three-day-old male neonate, a Nigerian of Ikwerre ethnicity, who was admitted with profuse bleeding from a soft tissue injury under the tongue, after clipping of the frenulum by a community health worker. He was severely pale and lethargic. He was resuscitated with intravenous fluids and a blood transfusion. The bleeding vessel was ligated with repair of the soft tissue. He also received antibiotics and was discharged home one week later. Conclusion Treatment of tongue-tie, a benign condition, when done by untrained personnel may result in life-threatening complications. Clinicians should pay more attention to parents' worries about this condition and give adequate counseling or refer them to trained personnel for surgical intervention where clinically indicated.

  4. Bone geometry, biomechanics, and mineral content of the distal radius in uremic patients with severe hyperparathyroidism; Valutazione delle proprieta' geometriche, biomeccaniche e osteodensitometriche del radio ultradistale mediante tomografia computerizzata quantitativa periferica nei pazienti uremici affetti da iperparatiroidismo grave

    Energy Technology Data Exchange (ETDEWEB)

    Di Leo, C.; Tarolo, G.L.; Bestetti, A.; Tagliabue, L.; Del Sole, A.; Aliberti, G. [Ospedale S. Paolo, Milan (Italy). Cattedra e Scuola di Specializzazione in Medicina Nucleare. Servizio di Medicina Nucleare; Ardemagni, A.; Gallieni, M.; Brancaccio, M.; Froehlich, W. [L' Accessorio nucleare (ACN). Laboratori scientifici, Cerro Maggiore, MI (Italy)

    1999-04-01

    Bone integrity and mineral status were studied with a noninvasive method in uremic patients with severe secondary hyperparathyroidism undergoing maintenance hemodialysis. [Italian] Gli autori propongono un metodo non invasivo per studiare la densita' minerale e le caratteristiche geometriche e biomeccaniche dell'osso nei pazienti uremici affetti da iperparatiroidismo secondario grave.

  5. Nutritional secondary hyperparathyroidism in two cats

    DEFF Research Database (Denmark)

    Dimopoulou, Maria; Kirpensteijn, Jolle; Nielsen, Dorte Hald

    2010-01-01

    severely affected cat, postmortem examination revealed changes consistent with nutritional secondary hyperparathyroidism and fibrous osteodystrophy, such as cortical thinning, massive connective tissue invasion in the diaphysis of long bones, and hypertrophy of the chief cells in both parathyroid glands...

  6. Neonatal screening for severe combined immunodeficiency in Brazil,

    Directory of Open Access Journals (Sweden)

    Marilia Pyles Patto Kanegae

    2016-08-01

    Full Text Available Abstract Objective To apply, in Brazil, the T-cell receptor excision circles (TRECs quantification technique using real-time polymerase chain reaction in newborn screening for severe combined immunodeficiency and assess the feasibility of implementing it on a large scale in Brazil. Methods 8715 newborn blood samples were collected on filter paper and, after DNA elution, TRECs were quantified by real-time polymerase chain reaction. The cutoff value to determine whether a sample was abnormal was determined by ROC curve analysis, using SSPS. Results The concentration of TRECs in 8,682 samples ranged from 2 to 2,181 TRECs/µL of blood, with mean and median of 324 and 259 TRECs/µL, respectively. Forty-nine (0.56% samples were below the cutoff (30 TRECs/µL and were reanalyzed. Four (0.05% samples had abnormal results (between 16 and 29 TRECs/µL. Samples from patients previously identified as having severe combined immunodeficiency or DiGeorge syndrome were used to validate the assay and all of them showed TRECs below the cutoff. Preterm infants had lower levels of TRECs than full-term neonates. The ROC curve showed a cutoff of 26 TRECs/µL, with 100% sensitivity for detecting severe combined immunodeficiency. Using this value, retest and referral rates were 0.43% (37 samples and 0.03% (3 samples, respectively. Conclusion The technique is reliable and can be applied on a large scale after the training of technical teams throughout Brazil.

  7. The Microcirculation Is Unchanged in Neonates with Severe Respiratory Failure after the Initiation of ECMO Treatment

    NARCIS (Netherlands)

    A.P.C. Top (Anke); E.A.B. Buijs (Erik ); M. van Dijk (Monique); D. Tibboel (Dick); C. Ince (Can)

    2012-01-01

    textabstractPurpose. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) is known to improve cardiorespiratory function and outcome in neonates with severe respiratory failure. We tested the hypothesis that VA-ECMO therapy improves the microcircu- lation in neonates with severe respiratory fa

  8. Consequences of long-term hyperparathyroidism.

    Science.gov (United States)

    Graal, M B; Wolffenbuttel, B H

    1998-07-01

    We describe a young woman with long-term untreated hyperparathyroidism with a superimposed vitamin D deficiency and an extremely decreased bone mineral density that was complicated by a vertebral fracture. Despite pretreatment with intravenous pamidronate and short-term vitamin D supplementation, severe and long-standing hypocalcaemia ('hungry bone syndrome') developed after parathyroidectomy. We discuss the consequences of hyperparathyroidism, especially the effects on bone, the complications of parathyroidectomy and the possibilities of preoperative treatment with bisphosphonates.

  9. Primary Hyperparathyroidism: An Overview

    OpenAIRE

    Jessica MacKenzie-Feder; Sandra Sirrs; Donald Anderson; Jibran Sharif; Aneal Khan

    2011-01-01

    Primary hyperparathyroidism is a common condition that affects 0.3% of the general population. Primary and tertiary care specialists can encounter patients with primary hyperparathyroidism, and prompt recognition and treatment can greatly reduce morbidity and mortality from this disease. In this paper we will review the basic physiology of calcium homeostasis and then consider genetic associations as well as common etiologies and presentations of primary hyperparathyroidism. We will consider...

  10. Primary Hyperparathyroidism: An Overview

    Directory of Open Access Journals (Sweden)

    Jessica MacKenzie-Feder

    2011-01-01

    Full Text Available Primary hyperparathyroidism is a common condition that affects 0.3% of the general population. Primary and tertiary care specialists can encounter patients with primary hyperparathyroidism, and prompt recognition and treatment can greatly reduce morbidity and mortality from this disease. In this paper we will review the basic physiology of calcium homeostasis and then consider genetic associations as well as common etiologies and presentations of primary hyperparathyroidism. We will consider emerging trends in detection and measurement of parathyroid hormone as well as available imaging modalities for the parathyroid glands. Surgical indications and approach will be reviewed as well as medical management of primary hyperparathyroidism with bisphosphonates and calcimimetics.

  11. Primary hyperparathyroidism in pregnancy.

    Science.gov (United States)

    Kamenický, Peter; Lecoq, Anne-Lise; Chanson, Philippe

    2016-06-01

    Primary hyperparathyroidism (PHPT) is one of the most common endocrine disorders in the general population but is rarely diagnosed during pregnancy. Symptoms of gestational PHPT may be unrecognized, or masked by physiological changes in calcium homeostasis associated with pregnancy. Gestational PHPT may have severe consequences for both mother and fetus. However, nowadays, gestational PHPT is usually diagnosed in earlier stages and milder forms, with low complication rates. Treatment should be individually tailored according to gestational age, the severity of hypercalcemia, and the risk-benefit balance. The conservative approach is preferred in mild forms, whereas surgery, usually performed during the second trimester, is reserved for symptomatic hypercalcemic PHPT. Given the young age of the patients, genetic causes should be considered.

  12. Osteosarcoma associated with hyperparathyroidism

    NARCIS (Netherlands)

    Jutte, PC; Rosso, R; de Paolis, M; Errani, C; Pasini, E; Campanacci, L; Bacci, G; Bertoni, F; Mercuri, M

    2004-01-01

    The fourth case in the literature is presented of a patient with the rare association of hyperparathyroidism and osteosarcoma. A 56-year-old woman presented with hyperparathyroidism and a lesion in the tibia. Initial diagnosis was brown tumor. Histology, however, revealed osteosarcoma, and the patie

  13. Osteosarcoma associated with hyperparathyroidism

    NARCIS (Netherlands)

    Jutte, PC; Rosso, R; de Paolis, M; Errani, C; Pasini, E; Campanacci, L; Bacci, G; Bertoni, F; Mercuri, M

    2004-01-01

    The fourth case in the literature is presented of a patient with the rare association of hyperparathyroidism and osteosarcoma. A 56-year-old woman presented with hyperparathyroidism and a lesion in the tibia. Initial diagnosis was brown tumor. Histology, however, revealed osteosarcoma, and the patie

  14. Osteosarcoma associated with hyperparathyroidism

    NARCIS (Netherlands)

    Jutte, PC; Rosso, R; de Paolis, M; Errani, C; Pasini, E; Campanacci, L; Bacci, G; Bertoni, F; Mercuri, M

    The fourth case in the literature is presented of a patient with the rare association of hyperparathyroidism and osteosarcoma. A 56-year-old woman presented with hyperparathyroidism and a lesion in the tibia. Initial diagnosis was brown tumor. Histology, however, revealed osteosarcoma, and the

  15. Apoptosis in Primary Hyperparathyroidism.

    Science.gov (United States)

    Segiet, Oliwia Anna; Mielańczyk, Łukasz; Piecuch, Adam; Michalski, Marek; Tyczyński, Szczepan; Brzozowa-Zasada, Marlena; Deska, Mariusz; Wojnicz, Romuald

    2017-03-31

    Primary hyperparathyroidism (PHPT) is defined by inappropriate elevation of parathormone, caused by parathyroid hyperplasia, also known as multi-gland disease (MGD), parathyroid adenoma (PA), or parathyroid carcinoma (PC). Although several studies have already been conducted, there is a lack of a definite diagnostic marker, which could unambiguously distinguish MGD from PA or PC. The accurate and prompt diagnosis has the key meaning for effective treatment and follow-up. This review paper presents the role of apoptosis in PHPT. The comparison of the expression of Fas, TRAIL, BCL-2 family members, p53 in MGD, PA, and PC, among others, was described. The expression of described factors varies among proliferative lesions of parathyroid gland; therefore, these could serve as additional markers to assist in the diagnosis.

  16. Cinacalcet Treatment of Primary Hyperparathyroidism

    OpenAIRE

    H. M. Rothe; Liangos, O.; Biggar, P.; Petermann, A.; Ketteler, M

    2011-01-01

    Although parathyroidectomy remains the only curative approach to most primary hyperparathyroidism cases, medical treatment with cinacalcet HCl has been proven to be a reasonable alternative for several patient subgroups. Cinacalcet almost always controls hypercalcemia and hypophosphatemia sufficiently. PTH levels are lowered, and cognitive parameters improve. While an increase in bone mineral density DEXA scan scores was not demonstrated in cinacalcet trials, the same applies to more than hal...

  17. Successful medical management of a neonate with spontaneous splenic rupture and severe hemophilia A.

    Science.gov (United States)

    Badawy, Sherif M; Rossoff, Jenna; Yallapragada, Sushmita; Liem, Robert I; Sharathkumar, Anjali A

    2017-03-01

    Splenic rupture in neonates is a rare event, usually occurring in the setting of underlying predisposing conditions. Here, we present the case of a term neonate who presented with worsening anemia in the setting of known hemolytic disease during the newborn period and was later found to have a spontaneous splenic rupture. He was subsequently diagnosed with severe hemophilia A, and was managed medically with recombinant factor VIII replacement therapy without any surgical intervention. This is the first reported case of a neonate who had spontaneous splenic rupture and severe hemophilia A, and underwent successful medical treatment without any surgical intervention.

  18. Levosimendan in a neonate with severe coarctation of aorta and low cardiac output syndrome

    Directory of Open Access Journals (Sweden)

    Yann Olivier Boegli

    2013-01-01

    Full Text Available We report successful use of levosimendan after failed balloon angioplasty in a critically ill neonate with coarctation of aorta (CoA and severe low cardiac output syndrome (LCOS. Treatment with levosimendan improved left heart function, and decreased lactate and brain natriuretic peptide levels. To our knowledge, this is the first report on the safe and successful use of levosimendan in the management of LCOS due to severe CoA in a neonate awaiting surgical repair.

  19. Neonate With Severe Heart Failure Related to Vein of Galen Malformation

    Directory of Open Access Journals (Sweden)

    Meng-Yu Chen

    2010-08-01

    Full Text Available We report a full-term female neonate who presented with respiratory distress and severe heart failure soon after birth. Heart failure secondary to perinatal infection was initially suspected. Subsequent echocardiography revealed aortic runoff, which led to consideration of an intracranial vascular abnormality. Ultrasound and magnetic resonance imaging of the brain confirmed a diagnosis of vein of Galen aneurismal malformation (VGAM. Endovascular coil embolization of the vascular anomaly was performed, resulting in improvement of heart failure. VGAM should be considered in the differential diagnosis of neonatal congestive heart failure with a structurally normal heart. Urgent endovascular embolization and aggressive medical treatment of heart failure improve prognosis in neonatal VGAM.

  20. Cholestasis and Hepatic Failure in a Neonate: A Case Report of Severe Pyruvate Kinase Deficiency.

    Science.gov (United States)

    Olivier, François; Wieckowska, Anna; Piedboeuf, Bruno; Alvarez, Fernando

    2015-11-01

    Unexpected severe cholestasis is part of the presentation in some neonates with hemolytic anemia but is usually self-resolving. Here we report the case of a neonate with pyruvate kinase deficiency (PKD) who presented severe hemolytic anemia at birth, characterized by a rapidly progressive and severe cholestasis with normal γ-glutamyl transpeptidase level associated with hepatic failure. After an extensive investigation to rule out contributing conditions explaining the severity of this patient's clinical presentation, PKD has remained the sole identified etiology. The patient abruptly died of sepsis at 3 months of age before a planned splenectomy and ongoing evaluation for liver transplantation. To the best of our knowledge, only a few similar cases of severe neonatal presentation of PKD complicated with severe hepatic failure and cholestasis have been reported.

  1. Subdural haemorrhage and severe coagulopathy resulting in transtentorial uncal herniation in a neonate undergoing therapeutic hypothermia

    OpenAIRE

    Wang, Dianna; McMillan, Hugh; Bariciak, Erika

    2014-01-01

    Therapeutic hypothermia has been shown to be efficacious for improving long-term neurodevelopmental outcomes following perinatal asphyxia. Thus, cooling protocols have been adopted at most tertiary neonatal centres. We present a case of a term neonate who underwent therapeutic whole-body cooling for hypoxic ischaemic encephalopathy following a difficult forceps delivery. She abruptly deteriorated, exhibiting signs of transtentorial uncal herniation and severe disseminated intravascular coagul...

  2. Image diagnosis of hyperparathyroidism

    OpenAIRE

    森川, 満; 佐賀, 裕司; 渡部, 嘉彦; 藤沢, 真; 金子, 茂男; 徳中, 荘平; 八竹, 直

    1989-01-01

    Between January, 1983 and January 1988, the diagnosis of hyperparathyroidism was made on 14 patients (8 primary and 6 secondary), and was identified operatively in all. The procedures for image diagnosis were computed tomography (CT), ultrasonography and scintigraphy. Scintigraphy was performed using Tc-99m and Tl-201 by a subtraction technique. For primary hyperparathyroidism, the accuracy of localization was 100% by CT, 87.5% by ultrasonography, 100% by scintigraphy, and 100% by the combina...

  3. ATYPICAL PRIMARY HYPERPARATHYROIDISM DUE TO HYPOVITAMINOSIS D.

    Science.gov (United States)

    Gaţu, Alina-Andreea; Velicescu, C; Novac, Roxana; Mogoş, Voichiţa; Brănişteanu, D D

    2015-01-01

    Vitamin D deficiency is nowadays very common in the general population and also in patients with primary hyperparathyroidism. Hypovitaminosis D may modify the clinical features and the severity of primary hyperparathyroidism. We present the case of a 75-year-old woman with a 10 year history of nephrolithiasis and severe osteoporosis, with multiple fragility fractures. Her bone and kidney status required a more thorough metabolic assessment. Despite minimal changes in serum calcium and phosphate levels, parathyroid hormone (PTH) level was markedly elevated. Ultrasound and specific Sesta-MIBI scintigraphy diagnosed and localized a left parathyroid adenoma. Vitamin D assessment showed levels in the range of hypovitaminosis. Vitamin D deficiency may mask hypercalcemia despite high serum PTH levels, and does not seem to diminish but on the contrary increases the risk of kidney lithiasis, as well as the deleterious effects of hyperparathyroidism on bone.

  4. [Severe neonatal acidosis: comparison and analysis of obstetrical practices in two French perinatal centers].

    Science.gov (United States)

    Pommereau-Lathelize, J; Maisonneuve, E; Jousse, M; Guilbaud, L; Carbonne, B; Pierre, F

    2014-04-01

    To identify and compare risk factors for severe neonatal acidosis, defined by an umbilical artery pH inferior to 7.00, and clinical practices in two different perinatal centers. In a retrospective study, from 2003 to 2008, in two university perinatal centers (Poitiers and Saint-Antoine in Paris) on all term pregnancies complicated by severe neonatal acidosis (umbilical artery pHacidosis. The rate was similar for both perinatal centers of Poitiers and Saint-Antoine (0.92% and 0.77% respectively). Factors associated with severe neonatal acidosis were similar in both centers: maternal age, thick meconium, prior cesarean section. There were differences in obstetrical practices between the two centers: there were more caesarean sections and assisted vaginal deliveries in Paris and more inductions of labour in Poitiers. Severe neonatal acidosis is associated with the geographical origin, the progress of labour and the mode of delivery. It seems that severe neonatal acidosis is unrelated to cesarean delivery. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  5. Effect of naloxone on level of plasma beta-endorphin in neonates with severe asphyxia

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: β-endorphin is the most actively endogenous substance of cerebral endorphin. When combined with opiate receptor specially, it manifests a strong morphine-like activity and can decrease sensitivity of central nervous system to carbon dioxide so as to inhibit breath.OBJECTIVE: To observe the changes of content of plasma β -endorphin in neonates with severe asphyxia after naloxone treatment in a large dosage.DESIGN: Randomized controlled observation.SETTINGS: Department of Pediatrics, Shenzhen Shajing People's Hospital; Center of Pediatrics,Guangzhou Zhujiang Hospital.PARTICIPANTS: A total of 97 neonates with severe asphyxia including 57 boys and 40 girls were selected from Neonatal Intensive Care Unit, Department of Pediatrics, Shenzhen Shajing People's Hospital from January 2004 to November 2005. Their gestational age was (38 ±3) weeks, body mass was (3.2± 1.7) kg,and hospitalization duration was (2.8±2.3) hours. All neonates met the diagnostic criteria of with severe asphyxia and all their parents provided the confirmed consent.METHODS: All neonates were treated with inspired oxygen, sedation, stopping terror, decreasing cranial pressure, maintaining a well blood perfusion and normal level of blood glucose (about 5.0 mmol/L). After hospitalization, 0.1 mg/(kg ·d) naloxone hydrochloride (Beijing Sihuan Pharmaceutical Technology Co., Ltd.;certification: H10900021; bullet preparation; 0.4 mg/ampoule) was intravenously dribbled into neonates for 4 - 6 hours, 14 days in total. 2 mL blood was collected from radial artery in neonates at the beginning of hospitalization and at 3 days after naloxone treatment, put in aprotinin-pre-cool tube, mixed evenly, and centrifuged at hypothermia. Plasma was maintained in refrigerator at - 70 ℃. The kit was provided by Neurobiology Department of Shanghai Second Military Medical University of Chinese PLA. Concentration of plasma β -endorphin was measured by using radio-immunity assay.All data were expressed as Mean

  6. Comparison between Calcitriol and Calcitriol Plus Low-Dose Cinacalcet for the Treatment of Moderate to Severe Secondary Hyperparathyroidism in Chronic Dialysis Patients

    Science.gov (United States)

    Lee, Yueh-Ting; Ng, Hwee-Yeong; Kuo, Chien-Chun; Chen, Te-Chuan; Wu, Chien-Shing; Chiu, Terry Ting-Yu; Lee, Wen-Chin; Lee, Chien-Te

    2013-01-01

    Aim: Uremic hyperparathyroidism (UHPT) has been shown to contribute to the development and progression of chronic kidney disease—mineral bone disorder. UHPT is frequently observed in chronic dialysis patients, and patients with UHPT are associated with increased risk of all-cause and cardiovascular mortality. Cinacalcet is a novel agent that increases sensitivity to the calcium-sensing receptor and is approved for control of UHPT. Nevertheless, cinacalcet is costly and information regarding efficacy of low-dose cinacalcet on UHPT is limited. Methods: We conducted a retrospective study to evaluate treatment with either low-dose calcitriol combined with low-dose cinacalcet (25 mg) (d-Cinacalcet) or calcitriol alone (VitD) in dialysis patients with moderate to severe UHPT. A total of 81 dialysis patients were enrolled (40 subjects in d-Cinacalcet group and 41 subjects in VitD group). Demographic data including age, gender, duration on dialysis and biochemical data were reviewed and recorded. Results: At the end of the study, the intact parathyroid hormone (iPTH) levels of the d-Cinacalcet group declined significantly (from 1166.0 ± 469.3 pg/mL to 679.8 ± 421.6 pg/mL, p < 0.0001), while there was no significant change in the VitD group. Significant decrease of serum calcium (Ca: 9.9 ± 0.6 mg/dL vs. 9.6 ± 0.8 mg/dL, p = 0.002), phosphorus (P: 5.9 ± 1.3 mg/dL vs. 4.9 ± 0.9 mg/dL, p < 0.0001) and calcium phosphate product (Ca × P: 58.7 ± 15.0 mg2/dL2 vs. 46.9 ± 8.9 mg2/dL2, p < 0.0001) were observed in the d-Cinacalcet group. In addition, the subjects in the d-Cinacalcet group had a greater proportion to achieve Kidney Disease Outcomes Quality Initiative (KDOQI)-recommended biochemical targets than the subjects in the VitD group (Ca: 48% vs. 24%; P: 78% vs. 32%; Ca × P: 85% vs. 37%; iPTH: 15% vs. 0%). Conclusions: We conclude that combination therapy of low-dose cinacalcet and calcitriol is more effective than calcitriol alone as a treatment for moderate and

  7. Comparison between Calcitriol and Calcitriol Plus Low-Dose Cinacalcet for the Treatment of Moderate to Severe Secondary Hyperparathyroidism in Chronic Dialysis Patients

    Directory of Open Access Journals (Sweden)

    Chien-Te Lee

    2013-04-01

    Full Text Available Aim: Uremic hyperparathyroidism (UHPT has been shown to contribute to the development and progression of chronic kidney disease—mineral bone disorder. UHPT is frequently observed in chronic dialysis patients, and patients with UHPT are associated with increased risk of all-cause and cardiovascular mortality. Cinacalcet is a novel agent that increases sensitivity to the calcium-sensing receptor and is approved for control of UHPT. Nevertheless, cinacalcet is costly and information regarding efficacy of low-dose cinacalcet on UHPT is limited. Methods: We conducted a retrospective study to evaluate treatment with either low-dose calcitriol combined with low-dose cinacalcet (25 mg (d-Cinacalcet or calcitriol alone (VitD in dialysis patients with moderate to severe UHPT. A total of 81 dialysis patients were enrolled (40 subjects in d-Cinacalcet group and 41 subjects in VitD group. Demographic data including age, gender, duration on dialysis and biochemical data were reviewed and recorded. Results: At the end of the study, the intact parathyroid hormone (iPTH levels of the d-Cinacalcet group declined significantly (from 1166.0 ± 469.3 pg/mL to 679.8 ± 421.6 pg/mL, p < 0.0001, while there was no significant change in the VitD group. Significant decrease of serum calcium (Ca: 9.9 ± 0.6 mg/dL vs. 9.6 ± 0.8 mg/dL, p = 0.002, phosphorus (P: 5.9 ± 1.3 mg/dL vs. 4.9 ± 0.9 mg/dL, p < 0.0001 and calcium phosphate product (Ca × P: 58.7 ± 15.0 mg2/dL2 vs. 46.9 ± 8.9 mg2/dL2, p < 0.0001 were observed in the d-Cinacalcet group. In addition, the subjects in the d-Cinacalcet group had a greater proportion to achieve Kidney Disease Outcomes Quality Initiative (KDOQI-recommended biochemical targets than the subjects in the VitD group (Ca: 48% vs. 24%; P: 78% vs. 32%; Ca × P: 85% vs. 37%; iPTH: 15% vs. 0%. Conclusions: We conclude that combination therapy of low-dose cinacalcet and calcitriol is more effective than calcitriol alone as a treatment for

  8. Prediction of severe neonatal hyperbilirubinemia using cord blood hydrogen peroxide: a prospective study.

    Directory of Open Access Journals (Sweden)

    Hung-Chieh Chou

    Full Text Available BACKGROUND: We hypothesized that cord blood hydrogen peroxide (H2O2 could be utilized to predict the severity of neonatal hyperbilirubinemia. METHODS: We prospectively enrolled term or near-term healthy neonates. Cord blood and capillary blood at three days of age were measured for hydrogen peroxide and bilirubin concentrations. For newborns with hyperbilirubinemia, further blood samples were obtained at five and seven days of age. Newborns were divided into severe or less severe hyperbilirubinemic groups (peak bilirubin ≥17 mg/dL or not. The sensitivity, specificity, and negative predictive values were determined. RESULTS: There were 158 neonates enrolled. The incidence of neonatal hyperbilirubinemia was 30.5% for a concentration ≥15 mg/dl. The rising patterns were similar among bilirubin concentrations and hydrogen peroxide levels during the first few days of life. There was a strong positive correlation between bilirubin concentrations and hydrogen peroxide levels after correlation analysis. The rate of severe hyperbilirubinemia was 13.3%. It revealed that a cord blood hydrogen peroxide signal level of 2500 counts/10 seconds was an appropriate cut-off for predicting severe hyperbilirubinemia. Sensitivity and the negative predictive value were 76.2% and 93.3%, respectively. CONCLUSIONS: Our findings confirm that hydrogen peroxide levels and bilirubin concentrations in cord and neonatal blood are closely related. A cord blood hydrogen peroxide level above 2500 counts/10 seconds associated with a high predictive value for severe hyperbilirubinemia. This method provides information about which neonate should be closely followed after discharge from the nursery.

  9. Severe anemia and hydrops in a neonate with parvovirus B19 infection: a case report

    Directory of Open Access Journals (Sweden)

    Negar Sajjadian

    2013-12-01

    Full Text Available Background: Anemia at the time of birth may cause some problem like asphyxia, heart failure shock or even death in a neonate. Different etiologies can be considered for this problem. Parvovirus B19, as a viral organism, can cause hydrops fetalis and neonatal anemia and consequent complications. We present here a case of newborn infant with severe anemia who had human parvovirus B19 infection.Case Presentation: A male newborn with gestational age of 36 week was born from a mother with poor prenatal care and history of contact with domestic animal. The neonate was very pale with Apgar score 2 at 1 min and received resuscitation, mechanical ventilation and repeated blood transfusion The hemoglobin level was significantly low. Analysis was made based on the clinical presentations. According to the case history, physical and laboratory findings, neonatal severe anemia induced by parvovirus B19 infection was suggested and Laboratory work up documented his infection with parovirus B19.Conclusion: Parvovirus B19 (B19 virus is the smallest single strand linear DNA virus in animal viruses, which is the only strain of parvovirus that is pathogenic in humans. Human parvovirus B19 may cross the placenta and result in fetal infection, morbidity and death. Parvovirus is an uncommon cause of neonatal anemia and hydrops fetalis so this etiology must be considered in differential diagnosis of anemia at birth.

  10. Neonatal circumcision in severe haemophilia: a survey of paediatric haematologists at United States Hemophilia Treatment Centers.

    Science.gov (United States)

    Kearney, S; Sharathkumar, A; Rodriguez, V; Chitlur, M; Valentino, L; Boggio, L; Gill, J

    2015-01-01

    Neonatal circumcision in patients with severe haemophilia has not been well studied. We performed a survey of paediatric haematologists from Hemophilia Treatment Centers (HTC) across the United States to better understand the attitudes toward and management of neonatal circumcision in haemophilia patients. Response rate to our survey was 40% (n = 64/159). Thirty-eight percent of respondents (n = 24) said that they would allow this procedure in the newborn period but in many cases this was against medical advice. The most reported concern regarding neonatal circumcision in haemophilia patients was the risk of development of an inhibitor (n = 25; 39%) followed by the concern for bleeding (n = 22; 34%) and issues related to vascular access in the neonate (n = 11; 17%). All respondents recommended at least one preprocedure dose of factor replacement. Twenty-two percent (n = 14) of respondents did not use more than one dose of factor replacement but 32% (n = 21) used 1-2 postoperative doses. The remainder of paediatric haematologists surveyed recommended between 3-5 (16%; n = 10) and 6-10 (3%, n = 2) additional days postoperatively. There was wide variation in both techniques of circumcision as well as adjuvant haemostatic agents used. Only 22% of respondents said that they had an established protocol for management of circumcision in the newborn haemophilia patient. These survey results highlight the need for evidence-based guidelines regarding the optimal management of circumcision in neonates with severe haemophilia.

  11. Neonatal bronchial hyperresponsiveness precedes acute severe viral bronchiolitis in infants

    DEFF Research Database (Denmark)

    Chawes, Bo L K; Poorisrisak, Porntiva; Johnston, Sebastian L

    2012-01-01

    Respiratory syncytial virus and other respiratory tract viruses lead to common colds in most infants, whereas a minority develop acute severe bronchiolitis often requiring hospitalization. We hypothesized that such an excessive response to respiratory tract viral infection is caused by host factors...

  12. Normocalcemic primary hyperparathyroidism

    Science.gov (United States)

    Bilezikian, John P.; Silverberg, Shonni J.

    2011-01-01

    SUMMARY Primary hyperparathyroidism is a common disorder of mineral metabolism characterized by incompletely regulated, excessive secretion of parathyroid hormone from one or more of the parathyroid glands. The historical view of this disease describes two distinct entities marked by two eras. When primary hyperparathyroidism was first discovered about 80 years ago, it was always symptomatic with kidney stones, bone disease and marked hypercalcemia. With the advent of the multichannel autoanalyzer about 40 years ago, the clinical phenotype changed to a disorder characterized by mild hypercalcemia and the absence of classical other features of the disease. We may now be entering a 3rd era in the history of this disease in which patients are being discovered with normal total and ionized serum calcium concentrations but with parathyroid hormone levels that are consistently elevated. In this article, we describe this new entity, normocalcemic primary hyperparathyroidism, a forme fruste of the disease. PMID:20485897

  13. PHENOBARBITAL FOR THE PREVENTION OF INTRACRANIAL DAMAGE IN CHINESE NEONATES WITH SEVERE ASPHYXIA

    Institute of Scientific and Technical Information of China (English)

    陈惠金; 吴圣楣; 黄萍; 孙建华; 陈冠仪; 储凇雯

    2000-01-01

    Objective To evaluate the effect of phenobarbital on preventing intracranial damage and seizure in Chinese neonates with severe asphyxia. Methods A control trial was carried out in 60 Chinese neonates with severe asphyxia. 30 neonates received loading dose of phenobarbital on an average of 5.6h of age. Results The mean value of phenobarbital serum level obtained at the 4th day after birth was 21.9μg/ml. No significant difference was observed in the two groups in terms of birth weight, gestational age, Apgar scores, sex, etc. The imaging diagnosis within 3d of age in all subjects showed normal brain in 14, brain edema in 9 and intraventricular hemorrhage (IVH) in 7 in the treated group with a 53.3% of total complication rate, and normal brain in 5, brain edema in 15, IVH in 9 and subarachnoid hemorrhage (SAH) in 1 in the untreated group with a 83.3% of total complication rate. The incidence of intracranial damage in the untreated group was significantly higher than that in the treated group (83.3% vs 53.3%, P<0.05). In the treated group, 4 neonates with seizure symptom were effectively controlled soon, and none of the other 26 developed seizure. The period of seizure in the treated group was significantly shorter than that in the untreated group (P<0. 05). Other clinical symptoms were also sooner improved and no side effects were observed among the neonates treated with phenobarbital. Conclusion The incidence of postasphyxiated intracranial damage was obviously decreased, and seizure could be prophylactically intervened by phenobarbital. It is recommended that early application of preventive phenobarbitalin severely asphyxiated neonates is reasonable in reducing the incidence of intracranial lesions and subsequent seizures.

  14. Two cases of severe sepsis caused by Bacillus pumilus in neonatal infants

    NARCIS (Netherlands)

    Kimouli, Maria; Vrioni, Georgia; Papadopoulou, Magdalini; Koumaki, Vasiliki; Petropoulou, Dimitra; Gounaris, Antonios; Friedrich, Alexander W.; Tsakris, Athanassios

    Bacillus pumilus is an environmental contaminant that has been rarely associated with clinical infections. Here, two cases of severe sepsis caused by B. pumilus are described in two full-term neonates; one in a female infant with no factors predisposing her to infection and the other in a male

  15. Two cases of severe sepsis caused by Bacillus pumilus in neonatal infants

    NARCIS (Netherlands)

    Kimouli, Maria; Vrioni, Georgia; Papadopoulou, Magdalini; Koumaki, Vasiliki; Petropoulou, Dimitra; Gounaris, Antonios; Friedrich, Alexander W.; Tsakris, Athanassios

    2012-01-01

    Bacillus pumilus is an environmental contaminant that has been rarely associated with clinical infections. Here, two cases of severe sepsis caused by B. pumilus are described in two full-term neonates; one in a female infant with no factors predisposing her to infection and the other in a male infan

  16. Osteosarcoma associated with hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Jutte, Paul C. [Istituti Orthopedici Rizzoli, Via Pupilli 1, 40136, Bologna (Italy); University Medical Center Groningen, PO Box 30.001, 9700 RB, Groningen (Netherlands); Rosso, Renato [Anatomia Patologica Universita di Pavia, via Forlanini 16, 27100, Pavia (Italy); Paolis, Massimiliano de; Errani, Costatino; Pasini, Elisabetta; Campanacci, Laura; Bacci, Gaetano; Bertoni, Franco; Mercuri, Mario [Istituti Orthopedici Rizzoli, Via Pupilli 1, 40136, Bologna (Italy)

    2004-08-01

    The fourth case in the literature is presented of a patient with the rare association of hyperparathyroidism and osteosarcoma. A 56-year-old woman presented with hyperparathyroidism and a lesion in the tibia. Initial diagnosis was brown tumor. Histology, however, revealed osteosarcoma, and the patient was treated accordingly. The experimental induction of osteosarcoma by parathormone in rodent studies makes this finding alarming, considering the increasing use of parathormone in the treatment of osteoporosis. The mechanism by which osteosarcoma is induced in humans cannot be explained based on current knowledge of mechanisms of action of parathyroid hormone. (orig.)

  17. Severe neonatal Marfan syndrome resulting from a de novo 3-bp insertion into the fibrillin gene on chromosome 15.

    OpenAIRE

    Milewicz, D M; Duvic, M

    1994-01-01

    Severe neonatal Marfan syndrome has features of the Marfan syndrome and congenital contractural arachnodactyly present at birth, along with unique features such as loose, redundant skin and pulmonary emphysema. Since the Marfan syndrome and congenital contractural arachnodactyly are due to mutations in different genes, it has been uncertain whether neonatal Marfan syndrome is due to mutations in the fibrillin gene on chromosome 15 or in another gene. We studied an infant with severe neonatal ...

  18. Severe anemia and hydrops in a neonate with parvovirus B19 infection: a case report

    OpenAIRE

    Negar Sajjadian; Ramin Jahadi

    2013-01-01

    Background: Anemia at the time of birth may cause some problem like asphyxia, heart failure shock or even death in a neonate. Different etiologies can be considered for this problem. Parvovirus B19, as a viral organism, can cause hydrops fetalis and neonatal anemia and consequent complications. We present here a case of newborn infant with severe anemia who had human parvovirus B19 infection.Case Presentation: A male newborn with gestational age of 36 week was born from a mother with poor pre...

  19. Severe acute haemorrhagic liver failure in a neonate with a favourable spontaneous outcome

    Energy Technology Data Exchange (ETDEWEB)

    Cavet, Madeleine; Balu, Marie; Garel, Catherine; Ducou le Pointe, Hubert [Universite Pierre et Marie Curie Paris VI, Service de Radiologie, Hopital d' enfants Armand-Trousseau, Paris (France); Mitanchez, Delphine; Alexandre, Marie [Universite Pierre et Marie Curie Paris VI, Service de Neonatologie, Hopital d' enfants Armand-Trousseau, Paris (France); Renolleau, Sylvain [Universite Pierre et Marie Curie Paris VI, Service de Reanimation, Hopital d' enfants Armand-Trousseau, Paris (France); Pariente, Daniele [Hopital de Bicetre, Service de Radiologie Pediatrique, Paris (France)

    2008-10-15

    Acute liver failure in neonates is rare and is frequently associated with an unfavourable outcome. There is no curative treatment other than liver transplantation. Screening for viral, metabolic, toxic or vascular disease is essential to assess the prognosis and to guide specific treatment. Hepatic haemorrhage in neonates is often associated with bacterial infection, trauma and coagulopathies. We present a unique case of neonatal acute liver failure and multifocal massive haemorrhagic intrahepatic lesions of traumatic origin, documented by US and MRI. The patient made a spontaneous recovery. Clinical, biological and imaging outcome was excellent despite the apparent severity of the initial features. The only possible aetiology was a difficult caesarean delivery for mild fetal macrosomia. (orig.)

  20. Incidence, Risk Factors and Causes of Severe Neonatal Hyperbilirubinemia in the South of Iran (Fars Province)

    Science.gov (United States)

    Najib, Khadije Sadat; Saki, Forough; Hemmati, Fariba; Inaloo, Soroor

    2013-01-01

    Background Today, Severe hyperbilirubinemia is the most common cause of neonatal readmissions. Identification of the cause of neonatal hyperbilirubinemia is useful in determining whether therapeutic interventions can prevent severe hyperbilirubinemia. Objectives We conducted this study to estimate the incidence of severe hyperbilirubinemia in Fars province and to determine the underlying causes and risk factors, which would be of value in identifying and implementing strategies to prevent morbidity from this condition. Patients and Methods All infants less than 28 days referred due to severe indirect hyperbilirubinemia were included. Complete history, physical examination and lab work up were performed. This is a longitudinal prospective study in 2009-2010. Results More common causes of severe indirect hyperbilirubinemia were blood group incompatibility, G6PD deficiency, sepsis and unknown. Risk factors of severe hyperbilirubinemia were Male sex, previous siblings with severe hyperbilirubinemia, early discharge, NVD, Breast feeding and cultural background of mothers. Conclusions Our study showed severe neonate indirect hyperbilirubinemia is still prevalence in Fars province and ethnic and cultural background of the mothers was more effective than school education in preventing hyperbilirubinemia complication. PMID:23984010

  1. The long quest for neonatal screening for severe combined immunodeficiency.

    Science.gov (United States)

    Buckley, Rebecca H

    2012-03-01

    Early recognition of severe combined immunodeficiency (SCID) is a pediatric emergency because a diagnosis before live vaccines or nonirradiated blood products are given and before development of infections permits lifesaving unfractionated HLA-identical or T cell-depleted haploidentical hematopoietic stem cell transplantation, enzyme replacement therapy, or gene therapy. The need for newborn screening for this condition has been recognized for the past 15 years. However, implementation of screening required development of an assay for T-cell lymphopenia that could be performed on dried bloodspots routinely collected from newborn infants for the past 48 years. This was accomplished 6 years ago, and there have already been 7 successful pilot studies. A recommendation to add SCID to the routine newborn-screening panel was approved by the Secretary's Advisory Committee on Heritable Disorders of Newborns and Children in 2010 and was soon after approved by the Secretary of Health and Human Services. It is important for allergists, immunologists, and other health care providers to take an active role in promoting newborn screening for SCID and other T-lymphocyte abnormalities in their states. Even more important will be their roles in establishing accurate diagnoses for infants with positive screen results and in ensuring that they are given the best possible treatment.

  2. The Microcirculation Is Unchanged in Neonates with Severe Respiratory Failure after the Initiation of ECMO Treatment

    Directory of Open Access Journals (Sweden)

    Anke P. C. Top

    2012-01-01

    Full Text Available Purpose. Venoarterial extracorporeal membrane oxygenation (VA-ECMO is known to improve cardiorespiratory function and outcome in neonates with severe respiratory failure. We tested the hypothesis that VA-ECMO therapy improves the microcirculation in neonates with severe respiratory failure. Methods. This single-center prospective observational pilot study took place in an intensive care unit of a level III university children’s hospital. Twenty-one-term neonates, who received VA-ECMO treatment, were included. The microcirculation was assessed in the buccal mucosa, using Orthogonal Polarization Spectral imaging, within 24 hours before (T1 and within the first 24 hours after initiation of ECMO treatment (T2. Data were compared to data of a ventilated control group (=7. Results. At baseline (T1, median functional capillary density (FCD, microvascular flow index (MFI, and heterogeneity index (HI did not differ between the ECMO group and the control group. At T2 the median FCD was lower in the control group (median [range]: 2.4 [1.4–4.2] versus 4.3 [2.8–7.4] cm/cm2; P value <0.001. For MFI and HI there were no differences at T2 between the two groups. Conclusion. The perfusion of the microcirculation does not change after initiation of VA-ECMO treatment in neonates with severe respiratory failure.

  3. Severe tricuspid regurgitation 14 years after diagnosis of "transient neonatal tricuspid regurgitation"

    OpenAIRE

    Boshoff, D.; Mertens, L.; Gewillig, M

    2001-01-01

    A 14 year old girl presented with severe tricuspid regurgitation after she was diagnosed with "transient tricuspid regurgitation of the newborn". In the neonatal period she had presented with severe tricuspid regurgitation without an obvious underlying anatomical cause. This spontaneously regressed during the first months of life. She was dismissed from follow up at the age of 5 years after complete normalisation of the clinical and echocardiographic examination. The subsequent evolution and ...

  4. [Pathophysiology of secondary hyperparathyroidism.

    Science.gov (United States)

    Kawarazaki, Hiroo

    2017-01-01

    Secondary hyperparathyroidism(SHPT)is the result of a compensatory response of the calcium phosphate homeostatic mechanism. Vitamin D deficiency and chronic kidney disease, both representative pathophysiological causes of SHPT, have been related not only to skeletal disorders but also cardiovascular diseases, ADL and QOL. This relates the importance of SHPT as a pathological cause or marker of such states.

  5. [Parenteral calcimimetics for the treatment of secondary hyperparathyroidism.

    Science.gov (United States)

    Hamano, Naoto; Fukagawa, Masafumi

    2017-01-01

    The oral calcimimetics, cinacalcet, is reported to be effective on secondary hyperparathyroidism resistant to classical treatment like phosphate binders or vitamin D receptor activator. The problem that gastrointestinal adverse events cause poor adherence, drug discontinuation, and insufficient dose escalation remains unsolved. The novel injectable calcimimetic, etelcalcetide, is recently developed and is expected to reduce such adverse events and improve the therapeutic effects on moderate to severe secondary hyperparathyroidism. Further studies are needed to demonstrate the potential benefits of etelcalcetide compared to cinacalcet.

  6. Improving diagnostic recognition of primary hyperparathyroidism with machine learning.

    Science.gov (United States)

    Somnay, Yash R; Craven, Mark; McCoy, Kelly L; Carty, Sally E; Wang, Tracy S; Greenberg, Caprice C; Schneider, David F

    2017-04-01

    Parathyroidectomy offers the only cure for primary hyperparathyroidism, but today only 50% of primary hyperparathyroidism patients are referred for operation, in large part, because the condition is widely under-recognized. The diagnosis of primary hyperparathyroidism can be especially challenging with mild biochemical indices. Machine learning is a collection of methods in which computers build predictive algorithms based on labeled examples. With the aim of facilitating diagnosis, we tested the ability of machine learning to distinguish primary hyperparathyroidism from normal physiology using clinical and laboratory data. This retrospective cohort study used a labeled training set and 10-fold cross-validation to evaluate accuracy of the algorithm. Measures of accuracy included area under the receiver operating characteristic curve, precision (sensitivity), and positive and negative predictive value. Several different algorithms and ensembles of algorithms were tested using the Weka platform. Among 11,830 patients managed operatively at 3 high-volume endocrine surgery programs from March 2001 to August 2013, 6,777 underwent parathyroidectomy for confirmed primary hyperparathyroidism, and 5,053 control patients without primary hyperparathyroidism underwent thyroidectomy. Test-set accuracies for machine learning models were determined using 10-fold cross-validation. Age, sex, and serum levels of preoperative calcium, phosphate, parathyroid hormone, vitamin D, and creatinine were defined as potential predictors of primary hyperparathyroidism. Mild primary hyperparathyroidism was defined as primary hyperparathyroidism with normal preoperative calcium or parathyroid hormone levels. After testing a variety of machine learning algorithms, Bayesian network models proved most accurate, classifying correctly 95.2% of all primary hyperparathyroidism patients (area under receiver operating characteristic = 0.989). Omitting parathyroid hormone from the model did not

  7. The immunologic basis for severe neonatal herpes disease and potential strategies for therapeutic intervention.

    Science.gov (United States)

    Gantt, Soren; Muller, William J

    2013-01-01

    Herpes simplex viruses types 1 and 2 (HSV-1 and HSV-2) infect a large proportion of the world's population. Infection is life-long and can cause periodic mucocutaneous symptoms, but it only rarely causes life-threatening disease among immunocompetent children and adults. However, when HSV infection occurs during the neonatal period, viral replication is poorly controlled and a large proportion of infants die or develop disability even with optimal antiviral therapy. Increasingly, specific differences are being elucidated between the immune system of newborns and those of older children and adults, which predispose to severe infections and reflect the transition from fetal to postnatal life. Studies in healthy individuals of different ages, individuals with primary or acquired immunodeficiencies, and animal models have contributed to our understanding of the mechanisms that control HSV infection and how these may be impaired during the neonatal period. This paper outlines our current understanding of innate and adaptive immunity to HSV infection, immunologic differences in early infancy that may account for the manifestations of neonatal HSV infection, and the potential of interventions to augment neonatal immune protection against HSV disease.

  8. The Immunologic Basis for Severe Neonatal Herpes Disease and Potential Strategies for Therapeutic Intervention

    Directory of Open Access Journals (Sweden)

    Soren Gantt

    2013-01-01

    Full Text Available Herpes simplex viruses types 1 and 2 (HSV-1 and HSV-2 infect a large proportion of the world’s population. Infection is life-long and can cause periodic mucocutaneous symptoms, but it only rarely causes life-threatening disease among immunocompetent children and adults. However, when HSV infection occurs during the neonatal period, viral replication is poorly controlled and a large proportion of infants die or develop disability even with optimal antiviral therapy. Increasingly, specific differences are being elucidated between the immune system of newborns and those of older children and adults, which predispose to severe infections and reflect the transition from fetal to postnatal life. Studies in healthy individuals of different ages, individuals with primary or acquired immunodeficiencies, and animal models have contributed to our understanding of the mechanisms that control HSV infection and how these may be impaired during the neonatal period. This paper outlines our current understanding of innate and adaptive immunity to HSV infection, immunologic differences in early infancy that may account for the manifestations of neonatal HSV infection, and the potential of interventions to augment neonatal immune protection against HSV disease.

  9. Meta-analysis shows that infants who have suffered neonatal sepsis face an increased risk of mortality and severe complications.

    Science.gov (United States)

    Bakhuizen, Sabine E; de Haan, Timo R; Teune, Margreet J; van Wassenaer-Leemhuis, Aleid G; van der Heyden, Jantien L; van der Ham, David P; Mol, Ben Willem J

    2014-12-01

    Infants suffering from neonatal sepsis face an increased risk of early death and long-term neurodevelopmental delay. This paper analyses and summarises the existing data on short-term and long-term outcomes of neonatal sepsis, based on 12 studies published between January 2000 and 1 April 2012 and covering 3669 neonates with sepsis. Infants who have suffered neonatal sepsis face an increased risk of mortality and severe complications such as brain damage and, or, neurodevelopmental delay. ©2014 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  10. Effect of Severe Maternal Iron Deficiency Anemia on Neonatal Platelet Indices.

    Science.gov (United States)

    Basu, Sriparna; Kumar, Naveen; Srivastava, Ragini; Kumar, Ashok

    2015-12-01

    To evaluate the effect of maternal iron deficiency anemia (IDA) on fetal thrombopoiesis. In this prospective observational study, maternal and cord blood iron status parameters (serum iron, serum ferritin, total iron-binding capacity, and transferrin saturation), and platelet indices, such as, absolute platelet count (APC), mean platelet volume (MPV), platelet distribution width (PDW) and plateletcrit, were estimated in a convenient sample of 142 mothers with IDA (hemoglobin neonates at term gestation. Mothers with antenatal thrombocytopenia, infections, inflammatory conditions, pregnancy-induced hypertension and neonates with perinatal asphyxia, sepsis and congenital malformations were excluded. For statistical analysis, the IDA group was further subdivided into mild-to-moderate (hemoglobin 7-10.9 g/dl) and severe (hemoglobin anemia. Cord blood APC and PDW were comparable between non-anemic and mild-to-moderate anemic mothers (242,550 ± 54,320/μL vs. 235,260 ± 34,620/μL for APC and 16.2 ± 1.4 vs. 16.4 ± 1.8 fl for PDW, respectively), but in severe IDA group, cord blood APC and PDW were significantly lower (74,520 ± 12,380/μL and 17.8 ± 2.1 fl, respectively, p neonates had a platelet count Neonates born to mothers with severe IDA had moderate thrombocytopenia with increased PDW, though no change was observed in MPV and plateletcrit. Further studies should be carried out to identify the cause and consequences of this observation.

  11. Neonatal purpura fulminans in newborn with severe congenital protein C deficiency: Case report

    Directory of Open Access Journals (Sweden)

    Sultan A. Jafarri

    2017-07-01

    Full Text Available Neonatal purpura fulminans (PF is a rare, life-threatening condition, caused by congenital or acquired deficiencies of protein C or S. PF describes a clinico-pathological entity of dermal microvascular thrombosis associated with disseminated intravascular coagulation (DIC and perivascular hemorrhage occurring in the newborn period. Here we describe a newborn with PF due to severe congenital protein C deficiency. The lesions started 5 h after birth but the infant was brought to our emergency department 20 h later. The infant was admitted in neonatal intensive care unit (NICU and treated with fresh frozen plasma (FFP, enoxaparin along with other supportive cares. In spite of impressive improvement of skin lesions, coordination with numerous subspecialties and aggressive NICU support, the infant died one month after admission due to multiorgan failure and septicaemia.

  12. Hormonal disturbances due to severe and mild forms of congenital adrenal hyperplasia are already detectable in neonatal life

    DEFF Research Database (Denmark)

    Faurschou, Signe; Mouritsen, Annette; Johannsen, Trine H;

    2015-01-01

    AIM: National screening programmes for congenital adrenal hyperplasia now include measuring several adrenal metabolites using highly sensitive liquid chromatography-tandem mass spectrometry. The aim of this study was to compare neonatal hormonal profiles - whole blood concentrations of 17α-hydrox....... CONCLUSION: There were significant differences in neonatal hormonal profiles between all groups and controls. This confirms that hormonal disturbances are already detectable in both severe and mild forms of congenital adrenal hyperplasia in neonatal life.......AIM: National screening programmes for congenital adrenal hyperplasia now include measuring several adrenal metabolites using highly sensitive liquid chromatography-tandem mass spectrometry. The aim of this study was to compare neonatal hormonal profiles - whole blood concentrations of 17α...

  13. The effects of cinacalcet treatment on bone mineral metabolism, anemia parameters, left ventricular mass index and parathyroid gland volume in hemodialysis patients with severe secondary hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Dilek Torun

    2016-01-01

    Full Text Available The aim of this study was to investigate the effects of cinacalcet therapy on anemia parameters, bone mineral metabolism, left ventricular mass index (LVMI and parathyroid gland volume in hemodialysis (HD patients with secondary hyperparathyroidism. Twenty-five HD patients (M/F: 11/14, mean age: 45.2 ± 17.9 years, mean HD duration: 96.4 ± 32.7 months were included in this prospective pilot study. The indication to start calcimimetic therapy was persistent serum levels of parathyroid hormone (PTH >1000 pg/mL, refractory to intravenous (i.v. vitamin D and phosphate-binding therapy. The initial and one-year results of adjusted serum calcium (Ca +2 , phosphate (P, Ca × P product, PTH, hemoglobin (Hb and ferritin levels, transferrin saturation index (TSAT, median weekly erythropoietin (EPO dose, LVMI, and parathyroid volume by parathyroid ultrasonography were determined. There were no differences between pre- and post-treatment levels of serum Ca +2 (P = 0.853, P (P = 0.447, Ca × P product (P = 0.587, PTH (P = 0.273, ferritin (P = 0.153 and TSAT (P = 0.104. After 1 year of calcimimetic therapy, the Hb levels were significantly higher than the initial levels (P = 0.048. The weekly dose of EPO decreased with no statistical significance. The dose of cinacalcet was increased from 32.4 ± 12.0 to 60.0 ± 24.4 mg/day (P = 0.01. There were no differences between the pre- and post-treatment results regarding weekly vitamin D dose, parenteral iron dose, LVMI and parathyroid volume. The results of our study suggest that cinacalcet therapy might have an additional benefit in the control anemia in HD patients.

  14. The effects of cinacalcet treatment on bone mineral metabolism, anemia parameters, left ventricular mass index and parathyroid gland volume in hemodialysis patients with severe secondary hyperparathyroidism.

    Science.gov (United States)

    Torun, Dilek; Yildiz, Ismail; Micozkadioglu, Hasan; Nursal, Gul Nihal; Yigit, Fatma; Ozelsancak, Ruya

    2016-01-01

    The aim of this study was to investigate the effects of cinacalcet therapy on anemia parameters, bone mineral metabolism, left ventricular mass index (LVMI) and parathyroid gland volume in hemodialysis (HD) patients with secondary hyperparathyroidism. Twenty-five HD patients (M/F: 11/14, mean age: 45.2±17.9 years, mean HD duration: 96.4±32.7 months) were included in this prospective pilot study. The indication to start calcimimetic therapy was persistent serum levels of parathyroid hormone (PTH)>1000 pg/mL, refractory to intravenous (i.v.) vitamin D and phosphate-binding therapy. The initial and one-year results of adjusted serum calcium (Ca+2), phosphate (P), Ca×P product, PTH, hemoglobin (Hb) and ferritin levels, transferrin saturation index (TSAT), median weekly erythropoietin (EPO) dose, LVMI, and parathyroid volume by parathyroid ultrasonography were determined. There were no differences between pre- and post-treatment levels of serum Ca+2 (P=0.853), P (P=0.447), Ca×P product (P=0.587), PTH (P=0.273), ferritin (P=0.153) and TSAT (P=0.104). After 1 year of calcimimetic therapy, the Hb levels were significantly higher than the initial levels (P=0.048). The weekly dose of EPO decreased with no statistical significance. The dose of cinacalcet was increased from 32.4±12.0 to 60.0±24.4 mg/day (P=0.01). There were no differences between the pre- and post-treatment results regarding weekly vitamin D dose, parenteral iron dose, LVMI and parathyroid volume. The results of our study suggest that cinacalcet therapy might have an additional benefit in the control anemia in HD patients.

  15. Single and multiple gland disease in primary hyperparathyroidism

    NARCIS (Netherlands)

    H.J. Bonjer (Jaap)

    1992-01-01

    textabstractThe scope of this thesis is: To review diagnostic procedures in primary hyperparathyroidism To review localization studies of parathyroid glands in hyperparathyroidism primary To assess the optima! surgical hyperparathyroidism by studying the recurrent hyperparathyroidism treatment of pr

  16. Repeated severe neonatal hemolysis due to Rhesus isoimmunization in a pregnant woman.

    Science.gov (United States)

    Chen, Da-Chung; Chang, Yeing-Kuan; Chen, Wei-Hwa; Liu, Jah-Yao

    2002-05-01

    Rhesus (Rh) isoimmunization presenting as severe neonatal hemolytic disease is rare in RhD negative primigravidas of Chinese ethnicity. We report the case of a 32-year-old pregnant Taiwanese woman, RhD negative, who gave birth vaginally to two RhD-positive full-term fetuses 6 years apart. Antenatal follow-up was uneventful and there was no obvious fetal-maternal hemorrhage except at the performance of amniocentesis at the 19th week of the first pregnancy without anti-D immune globulin prophylaxis. Although anti-D immune globulins were administered to the mother within 1 hour after each birth, both of the newborns had severe neonatal hemolysis refractory to phototherapy and were rescued by exchange transfusions. Both of the children were well at age 7-years-old and one-year-old respectively In conclusion, with suspicion of fetal-maternal hemorrhage in RhD-negative pregnancies post amniocentesis, serial monitoring of indirect Coombs titer with appropriate management is mandatory.

  17. A study of factors that influence the severity of neonatal narcotic withdrawal.

    Science.gov (United States)

    Ostrea, E M; Chavez, C J; Strauss, M E

    1975-01-01

    1. History is unreliable in assessing maternal drug habit. Morphine was detected in significant amounts in maternal and fetal urine regardless of whether the mother was on a methadone program or whether she denied any use of heroin during the last trimester of pregnancy. 2. Infants born to drug-addicted mothers were, in general, of birthweight normal and appropriate for gestational age (i.e., greater that 10th percentile). The infants born to mothers on a methadone clinic program had a higher birthweight compared to those whose mothers were not on any methadone program. 3. In order of frequency, the signs and symptoms of withdrawal were: central nervous system manifestations-fist sucking, irritability, tremors, sneezing, high-pitch cry, hypertonia; vasomotor in the form of stuffy nose; and gastrointestinal in the form of sweating, diarrhea, vomiting and yawning. Convulsions were not noted. No death occurred. 4. The severity of neonatal narcotic withdrawal did not correlate with the infant's gestational age, APGAR, sex or race; nor with maternal age, parity, duration of heroin addiction or duration of methadone intake. Also, it did not correlate with the total morphine level measured either in infant's or mother's urine or in cord blood. The serum levels of calcium and glucose were normal and identical in either mild or severe withdrawal. 5. The severity of neonatal withdrawal correlated significantly with the methadone dose per day of the mother (in initial, final or average dose). A maternal methadone dose of more than 20 mg per day was associated with a higher incidence of moderate to severe withdrawal in their babies. As a corollary, it was also noted that infants whose mothers were on a high methadone dose (i.e., greater than 20 mg per day) had a greater postnatal weight loss despite a significantly higher birthweight initially, and stayed in the hospital longer. 6. Finally, the modification of the environment to reduce external stimuli to the infant born to a

  18. Severe neonatal anemia from fetomaternal hemorrhage: report from a multihospital health-care system.

    Science.gov (United States)

    Christensen, R D; Lambert, D K; Baer, V L; Richards, D S; Bennett, S T; Ilstrup, S J; Henry, E

    2013-06-01

    The incidence of fetomaternal hemorrhage that is severe enough to cause neonatal anemia is not known. Owing to its relative rarity, much of the literature describing this condition is in the form of case reports and small case series. We performed a large, muiticentered, sequential, case series to determine the incidence, antecedents and outcomes. From the multicentered databases of Intermountain Healthcare, we obtained records of all neonates with hematocrit (Hct) anemia with evidence of fetomaternal hemorrhage (incidence estimate, 1 per 9160 live births). The initial Hgb ranged from 1.4 to 10.2 g dl(-1) (Hct 29.8%). The initial Hgb was neonatal Hgb was <3 g dl(-1) had reported absent fetal movement, as did 13 of 18 mothers when the initial Hgb was <7 g dl(-1). Outcomes were poorer in those with the lowest initial Hgb; in the two lowest, one died on day 1, and the other developed a grade 4 intraventricular hemorrhage (IVH). The adverse outcomes of death, IVH, periventricular leukomalacia, bronchopulmonary dysplasia or hypoxic-ischemic encephalopathy were common; occurring in 71% (17 of the 24), including all with an initial Hgb <5 g dl(-1) and all born at ≤35 weeks of gestation. Fetomaternal hemorrhage is a rare but sometimes devastating condition. Those with fetomaternal hemorrhage and an initial Hgb of <5 g dl(-1) are expected to need resuscitation at birth, to receive emergent transfusion support and to be at risk for death and major morbidities. Antenatal suspicion of this diagnosis should occur when absent fetal movement is reported. Improvements in rapid diagnosis are needed to prepare first responders and transfusion services.

  19. Severe congenital rickets secondary to maternal hypovitaminosis D: a case report.

    Science.gov (United States)

    Maiyegun, Sitratallah Olawunmi; Malek, Abdul Hakeem; Devarajan, Lakshmi Viswanathan; Dahniya, Mohammed Haroun

    2002-06-01

    We report a full-term baby boy who presented soon after birth with severe congenital rickets. Maternal and neonatal vitamin D levels were very low and the infant responded well to oral vitamin D. Transient secondary hyperparathyroidism normalised on treatment. The mother's vitamin D deficiency was attributed to the region's cultural dress code which prevents exposure to sunlight. There has not been a previous report of severe congenital rickets from this region.

  20. Bone disease in primary hyperparathyroidism

    Science.gov (United States)

    Bandeira, Francisco; Cusano, Natalie E.; Silva, Barbara C.; Cassibba, Sara; Almeida, Clarissa Beatriz; Machado, Vanessa Caroline Costa; Bilezikian, John P.

    2015-01-01

    Bone disease in severe primary hyperparathyroidism (PHPT) is described classically as osteitis fibrosa cystica (OFC). Bone pain, skeletal deformities and pathological fractures are features of OFC. Bone mineral density is usually extremely low in OFC, but it is reversible after surgical cure. The signs and symptoms of severe bone disease include bone pain, pathologic fractures, proximal muscle weakness with hyperreflexia. Bone involvement is typically characterized as salt-and-pepper appearance in the skull, bone erosions and bone resorption of the phalanges, brown tumors and cysts. In the radiography, diffuse demineralization is observed, along with pathological fractures, particularly in the long bones of the extremities. In severe, symptomatic PHPT, marked elevation of the serum calcium and PTH concentrations are seen and renal involvement is manifested by nephrolithiasis and nephrocalcinosis. A new technology, recently approved for clinical use in the United States and Europe, is likely to become more widely available because it is an adaptation of the lumbar spine DXA image. Trabecular bone score (TBS) is a gray-level textural analysis that provides an indirect index of trabecular microarchitecture. Newer technologies, such as high-resolution peripheral quantitative computed tomography (HR-pQCT), have provided further understanding of the microstructural skeletal features in PHPT. PMID:25166047

  1. Hyperparathyroidism of Renal Disease

    Science.gov (United States)

    Yuen, Noah K; Ananthakrishnan, Shubha; Campbell, Michael J

    2016-01-01

    Renal hyperparathyroidism (rHPT) is a common complication of chronic kidney disease characterized by elevated parathyroid hormone levels secondary to derangements in the homeostasis of calcium, phosphate, and vitamin D. Patients with rHPT experience increased rates of cardiovascular problems and bone disease. The Kidney Disease: Improving Global Outcomes guidelines recommend that screening and management of rHPT be initiated for all patients with chronic kidney disease stage 3 (estimated glomerular filtration rate, < 60 mL/min/1.73 m2). Since the 1990s, improving medical management with vitamin D analogs, phosphate binders, and calcimimetic drugs has expanded the treatment options for patients with rHPT, but some patients still require a parathyroidectomy to mitigate the sequelae of this challenging disease. PMID:27479950

  2. Prolonged thrombocytopenia in a child with severe neonatal alloimmune reaction and Noonan syndrome.

    Science.gov (United States)

    Salva, Inês; Batalha, Sara; Maia, Raquel; Kjollerstrom, Paula

    2016-06-01

    Fetomaternal alloimmune thrombocytopenia (FMAIT) caused by maternal antibodies is the leading cause of severe neonatal thrombocytopenia. A 1-month-old Caucasian girl was referred to our Hematology Clinic for persistent thrombocytopenia diagnosed after a bleeding episode. Diagnostic tests suggested FMAIT. Mild thrombocytopenia persisted for 18 months, and subsequent findings of dysmorphic facies, short stature and mild pulmonary stenosis led to the hypothesis of Noonan syndrome (NS), which was confirmed by genetic test. Other hematological abnormalities were excluded and she had no further bleeding episodes. This case illustrates the possibility of different diagnoses with the same clinical manifestations. The persistence of thrombocytopenia longer than expected associated with typical physical features led to the diagnosis of NS.

  3. Severe Hemorrhage from the Umbilical Cord at Birth: A Preventable Cause of Neonatal Shock

    Directory of Open Access Journals (Sweden)

    Neetu Singh

    2013-01-01

    Full Text Available Posthemorrhagic anemia is a rare but important cause of anemia in neonates, second only to hemolytic anemia of newborn. Most cases of posthemorrhagic anemia are reported from fetomaternal hemorrhage or umbilical cord accidents in utero. This case report describes a preterm infant who developed severe anemia and shock immediately after delivery related to an acute hemorrhage through patent umbilical cord vessels secondary to a tear in the umbilical cord at the site of cord clamping. We believe that umbilical cord bleeding from errors in cord clamping could be an important cause of acute blood loss in the delivery room and that it may result in significant clinical morbidity, especially in extremely premature infants.

  4. Mineralization defect but no effect on hypercalcemia during clodronate treatment in secondary hyperparathyroidism.

    Science.gov (United States)

    Ring, T; Sodemann, B; Nielsen, C; Melsen, F; Kornerup, H J

    1995-09-01

    In four patients with severe secondary hyperparathyroidism, treatment with clodronate caused no decrease in serum calcium. In one of the patients treatment for seven months was associated with a severe mineralization defect which was not caused by aluminium. This lesion was reversible upon termination of clodronate treatment. In a single patient without hyperparathyroidism, a precipitous decrease in serum calcium was observed due to clodronate. However, long-term treatment with clodronate did not ameliorate ectopic calcification in this patient. It is concluded that in severe secondary hyperparathyroidism, clodronate does not always decrease serum calcium. Our experience suggest that clodronate like other bisphosphonates may inhibit bone mineralization.

  5. Unreported births and deaths, a severe obstacle for improved neonatal survival in low-income countries; a population based study

    Directory of Open Access Journals (Sweden)

    Wallin Lars

    2008-03-01

    Full Text Available Abstract Background In order to improve child survival there is a need to target neonatal mortality. In this pursuit, valid local and national statistics on child health are essential. We analyze to what extent births and neonatal deaths are unreported in a low-income country and discuss the consequences at local and international levels for efforts to save newborn lives. Methods Information on all births and neonatal deaths in Quang Ninh province in Northern Vietnam in 2005 was ascertained by systematic inventory through group interviews with key informants, questionnaires and examination of health facility records. Health care staff at 187 Community Health Centers (CHC and 18 hospitals, in addition to 1372 Village Health Workers (VHW, were included in the study. Results were compared with the official reports of the Provincial Health Bureau. Results The neonatal mortality rate (NMR was 16/1000 (284 neonatal deaths/17 519 births, as compared to the official rate of 4.2/1000. The NMR varied between 44/1000 and 10/1000 in the different districts of the province. The under-reporting was mainly attributable to a dysfunctional reporting system and the fact that families, not the health system, were made responsible to register births and deaths. This under-reporting has severe consequences at local, national and international levels. At a local level, it results in a lack of awareness of the magnitude and differentials in NMR, leading to an indifference towards the problem. At a national and international level the perceived low mortality rate is manifested in a lack of investments in perinatal health programs. Conclusion This example of a faulty health information system is reportedly not unique in low and middle income countries where needs for neonatal health reforms are greatest. Improving reporting systems on births and neonatal deaths is a matter of human rights and a prerequisite for reducing neonatal mortality in order to reach the fourth

  6. [Prevention of severe diseases in apparently healthy newborn infants by new neonatal diagnostic methods].

    Science.gov (United States)

    Velázquez, A

    1999-02-01

    The benefit of Tamiz Neonatal Ampliado is very high in relation with its cost, which is reasonably low. The Society for Inherited Metabolic Disorders, which is the main association in the world dealing with these diseases, has recently urged the responsible ones for neonatal health, so they increase their programs about Tamiz Neonatal (personal communication), as much as possible; its use should cover all new borns.

  7. Hyperparathyroidism and psychosis: possible prelude to murder.

    Science.gov (United States)

    Bresler, S A; Logan, W S; Washington, D

    2000-05-01

    The authors present a case of a middle aged attorney who suffered from hyperparathyroidism and a psychotic disorder. It is possible that the hyperparathyroidism may have precipitated an acute psychotic delusional rage leading to an attempted mass murder. They discuss the relationship between hyperparathyroidism and neuropsychiatric symptoms in consideration of available research.

  8. Rare Skeletal Complications in the Setting of Primary Hyperparathyroidism

    Science.gov (United States)

    Sabanis, Nikos; Gavriilaki, Eleni; Paschou, Eleni; Kalaitzoglou, Asterios; Papanikolaou, Dimitrios; Ioannidou, Pinelopi; Vasileiou, Sotirios

    2015-01-01

    Parathyroid carcinoma represents an extremely rare neoplasm with diverse clinical manifestations which vary from asymptomatic patients to severe complications of hypercalcemia or parathyrotoxicosis while skeletal involvement is rather common. Herein we aimed at presenting a unique case of a young patient with rare aggressive skeletal complications of parathyroid cancer that initially were misdiagnosed. Ossification of the cervical ligamentum flavum and skull tumor illustrates erosive bonny lesions of hyperparathyroidism that in association with previous medical history of recurrent nephrolithiasis and biochemical findings guide the diagnosis. We suggest that increased awareness and holistic approach are needed in order to recognize and further investigate signs and symptoms of hyperparathyroidism. PMID:26664767

  9. Rare Skeletal Complications in the Setting of Primary Hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Nikos Sabanis

    2015-01-01

    Full Text Available Parathyroid carcinoma represents an extremely rare neoplasm with diverse clinical manifestations which vary from asymptomatic patients to severe complications of hypercalcemia or parathyrotoxicosis while skeletal involvement is rather common. Herein we aimed at presenting a unique case of a young patient with rare aggressive skeletal complications of parathyroid cancer that initially were misdiagnosed. Ossification of the cervical ligamentum flavum and skull tumor illustrates erosive bonny lesions of hyperparathyroidism that in association with previous medical history of recurrent nephrolithiasis and biochemical findings guide the diagnosis. We suggest that increased awareness and holistic approach are needed in order to recognize and further investigate signs and symptoms of hyperparathyroidism.

  10. Cinacalcet Treatment of Primary Hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    H. M. Rothe

    2011-01-01

    Full Text Available Although parathyroidectomy remains the only curative approach to most primary hyperparathyroidism cases, medical treatment with cinacalcet HCl has been proven to be a reasonable alternative for several patient subgroups. Cinacalcet almost always controls hypercalcemia and hypophosphatemia sufficiently. PTH levels are lowered, and cognitive parameters improve. While an increase in bone mineral density DEXA scan scores was not demonstrated in cinacalcet trials, the same applies to more than half of patients after parathyroidectomy. Medical therapy should be first choice in patients with hyperplasia in all glands rather than an isolated adenoma (10–15%, patients with persisting HPT following unsuccessful surgery or inoperable cases due to comorbidities, and patients detected in lab screens for hypercalcemia before developing symptoms who should be treated early but are usually reluctant to undergo surgery. Nephrolithiasis was not found to occur more frequently in cinacalcet trial groups, but urine calcium excretion as one major risk factor of this complication of primary HPT may increase on cinacalcet. Patients carrying the rs1042636 polymorphism of the calcium-sensing receptor gene respond more sensitively to cinacalcet and have a higher risk of calcium stone formation. Cinacalcet is usually administered twice daily but three or four doses per day should be discussed to mimic the beneficial pulsatile PTH-pattern.

  11. Cinacalcet Treatment of Primary Hyperparathyroidism

    Science.gov (United States)

    Rothe, H. M.; Liangos, O.; Biggar, P.; Petermann, A.; Ketteler, M.

    2011-01-01

    Although parathyroidectomy remains the only curative approach to most primary hyperparathyroidism cases, medical treatment with cinacalcet HCl has been proven to be a reasonable alternative for several patient subgroups. Cinacalcet almost always controls hypercalcemia and hypophosphatemia sufficiently. PTH levels are lowered, and cognitive parameters improve. While an increase in bone mineral density DEXA scan scores was not demonstrated in cinacalcet trials, the same applies to more than half of patients after parathyroidectomy. Medical therapy should be first choice in patients with hyperplasia in all glands rather than an isolated adenoma (10–15%), patients with persisting HPT following unsuccessful surgery or inoperable cases due to comorbidities, and patients detected in lab screens for hypercalcemia before developing symptoms who should be treated early but are usually reluctant to undergo surgery. Nephrolithiasis was not found to occur more frequently in cinacalcet trial groups, but urine calcium excretion as one major risk factor of this complication of primary HPT may increase on cinacalcet. Patients carrying the rs1042636 polymorphism of the calcium-sensing receptor gene respond more sensitively to cinacalcet and have a higher risk of calcium stone formation. Cinacalcet is usually administered twice daily but three or four doses per day should be discussed to mimic the beneficial pulsatile PTH-pattern. PMID:21461394

  12. Continuous inhaled iloprost in a neonate with d-transposition of the great arteries and severe pulmonary arterial hypertension.

    Science.gov (United States)

    Dykes, John C; Torres, Marilyn; Alexander, Plato J

    2016-03-01

    This report describes the case of a neonate with d-transposition of the great arteries and severe pulmonary arterial hypertension stabilised in the post-operative period with continuous iloprost nebulisation. To our knowledge, this is the first documented method of treating post-operative severe pulmonary arterial hypertension with continuous inhaled iloprost in a patient with complex CHD. We found this method of delivering the drug very effective in stabilising haemodynamic swings in the setting of severe pulmonary arterial hypertension.

  13. Primary hyperparathyroidism in pregnancy.

    Science.gov (United States)

    Rchachi, Meryem; El Ouahabi, Hanan; Boujraf, Saïd; Ajdi, Farida

    2017-01-01

    Primary hyperparathyroidism (PHT) is a frequent and asymptomatic pathology in 80% of patients, and a parathyroidal adenoma is found in the most case. However, rare cases might occur during pregnancy; and the literature reported up to 200 cases. We report three cases of PHT that occurred during the second and last third trimesters of pregnancy stage in patients aged 26, 42 and 32-year-old. Despite diagnosis limitations, since scintigraphy is prohibited in pregnancy, the patients were managed conservatively with good prognosis for both mothers and babies. L'hyperparathyroïdie primaire (HTP) est une maladie fréquente et asymptomatique chez 80% des patients. Un adénome parathyroïdien est retrouvé chez une grande majorité des cas. Cependant, l'apparition au cours de la grossesse est particulièrement rare et la littérature ne rapporte que 200 cas. Nous en rapportons 3 cas d'hyperparathyroïdie âgés de 26, 42 et 32 ans, survenue au cours du deuxième et troisième trimestre de la grossesse. Malgré les limites diagnostiques dues à l'interdiction de la scintigraphie au cours de la grossesse, la gestion avait été faite de façon très conservative de sorte à avoir le meilleur pronostic pour les patientes leurs fétus.

  14. Renal secondary hyperparathyroidism in dogs.

    Science.gov (United States)

    Stillion, Jenefer R; Ritt, Michelle G

    2009-06-01

    The parathyroid glands secrete parathyroid hormone (PTH), which is important for maintaining calcium homeostasis. Parathyroid gland hyperplasia and subsequent hyperparathyroidism can occur secondary to chronic renal failure in dogs, resulting in significant alterations in calcium metabolism. Renal secondary hyperparathyroidism is a complex, multifactorial syndrome that involves changes in circulating levels of calcium, PTH, phosphorus, and 1,25-dihydroxycholecalciferol (calcitriol). An increased PTH level can have deleterious effects, including soft tissue mineralization, fibrous osteodystrophy, bone marrow suppression, urolithiasis, and neuropathy. Dietary phosphorus restriction, intestinal phosphate binders, and calcitriol supplementation may slow the progression of renal disease and decrease PTH concentrations in animals with secondary hyperparathyroidism; however, the prognosis for these animals is guarded to poor.

  15. Biallelic Mutations in LIPT2 Cause a Mitochondrial Lipoylation Defect Associated with Severe Neonatal Encephalopathy.

    Science.gov (United States)

    Habarou, Florence; Hamel, Yamina; Haack, Tobias B; Feichtinger, René G; Lebigot, Elise; Marquardt, Iris; Busiah, Kanetee; Laroche, Cécile; Madrange, Marine; Grisel, Coraline; Pontoizeau, Clément; Eisermann, Monika; Boutron, Audrey; Chrétien, Dominique; Chadefaux-Vekemans, Bernadette; Barouki, Robert; Bole-Feysot, Christine; Nitschke, Patrick; Goudin, Nicolas; Boddaert, Nathalie; Nemazanyy, Ivan; Delahodde, Agnès; Kölker, Stefan; Rodenburg, Richard J; Korenke, G Christoph; Meitinger, Thomas; Strom, Tim M; Prokisch, Holger; Rotig, Agnes; Ottolenghi, Chris; Mayr, Johannes A; de Lonlay, Pascale

    2017-08-03

    Lipoate serves as a cofactor for the glycine cleavage system (GCS) and four 2-oxoacid dehydrogenases functioning in energy metabolism (α-oxoglutarate dehydrogenase [α-KGDHc] and pyruvate dehydrogenase [PDHc]), or amino acid metabolism (branched-chain oxoacid dehydrogenase, 2-oxoadipate dehydrogenase). Mitochondrial lipoate synthesis involves three enzymatic steps catalyzed sequentially by lipoyl(octanoyl) transferase 2 (LIPT2), lipoic acid synthetase (LIAS), and lipoyltransferase 1 (LIPT1). Mutations in LIAS have been associated with nonketotic hyperglycinemia-like early-onset convulsions and encephalopathy combined with a defect in mitochondrial energy metabolism. LIPT1 deficiency spares GCS deficiency and has been associated with a biochemical signature of combined 2-oxoacid dehydrogenase deficiency leading to early death or Leigh-like encephalopathy. We report on the identification of biallelic LIPT2 mutations in three affected individuals from two families with severe neonatal encephalopathy. Brain MRI showed major cortical atrophy with white matter abnormalities and cysts. Plasma glycine was mildly increased. Affected individuals' fibroblasts showed reduced oxygen consumption rates, PDHc, α-KGDHc activities, leucine catabolic flux, and decreased protein lipoylation. A normalization of lipoylation was observed after expression of wild-type LIPT2, arguing for LIPT2 requirement in intramitochondrial lipoate synthesis. Lipoic acid supplementation did not improve clinical condition nor activities of PDHc, α-KGDHc, or leucine metabolism in fibroblasts and was ineffective in yeast deleted for the orthologous LIP2. Copyright © 2017 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  16. Primary Hyperparathyroidism with Extensive Brown Tumors and Multiple Fractures in a 20-Year-Old Woman

    Science.gov (United States)

    Choi, Ju Hee; Kim, Kyoung Jin; Lee, Ye Jin; Kim, Sun Hwa; Kim, Sin Gon; Jung, Kwang Yoon; Choi, Dong Seop

    2015-01-01

    A brown tumor is a benign fibrotic, erosive bony lesion caused by localized, rapid osteoclastic turnover, resulting from hyperparathyroidism. Although brown tumors are one of the most pathognomonic signs of primary hyperparathyroidism, they are rarely seen in clinical practice. In this report, we present a case of 20-year-old woman with recurrent fractures and bone pain. Plain digital radiographs of the affected bones revealed multiple erosive bone tumors, which were finally diagnosed as brown tumors associated with primary hyperparathyroidism due to a parathyroid adenoma. This case shows that multiple, and clinically severe form of brown tumors can even occur in young patients. PMID:26354493

  17. Primary Hyperparathyroidism with Extensive Brown Tumors and Multiple Fractures in a 20-Year-Old Woman

    Directory of Open Access Journals (Sweden)

    Ju Hee Choi

    2015-12-01

    Full Text Available A brown tumor is a benign fibrotic, erosive bony lesion caused by localized, rapid osteoclastic turnover, resulting from hyperparathyroidism. Although brown tumors are one of the most pathognomonic signs of primary hyperparathyroidism, they are rarely seen in clinical practice. In this report, we present a case of 20-year-old woman with recurrent fractures and bone pain. Plain digital radiographs of the affected bones revealed multiple erosive bone tumors, which were finally diagnosed as brown tumors associated with primary hyperparathyroidism due to a parathyroid adenoma. This case shows that multiple, and clinically severe form of brown tumors can even occur in young patients.

  18. Emerging biomarkers for the diagnosis of severe neonatal infections applicable to low-resource settings

    Directory of Open Access Journals (Sweden)

    Thor A. Wagner

    2011-12-01

    Full Text Available More than 500 000 children die each year in low resource settings due to serious neonatal infections. Better diagnostics that can be utilized in these settings to identify infected infants has the potential to significantly reduce neonatal deaths and the associated morbidity. A systematic review was performed and identified more than 250 potential new biomarkers for the diagnosis of serious neonatal infections. Eight of these biomarkers were both high-performance and high-abundance (antithrombin, inter-a inhibitor proteins, interferon-g inducible protein-10, interleukin-1 receptor antagonist, LPS binding protein, mannose binding lectin, serum amyloid A, resistin, visfatin, and are promising for the diagnosis of serious neonatal infections in low resource settings. Future clinical trials comparing these biomarkers with more traditional biomarkers seem warranted.

  19. Severe neonatal Marfan syndrome resulting from a de novo 3-bp insertion into the fibrillin gene on chromosome 15.

    Science.gov (United States)

    Milewicz, D. M.; Duvic, M.

    1994-01-01

    Severe neonatal Marfan syndrome has features of the Marfan syndrome and congenital contractural arachnodactyly present at birth, along with unique features such as loose, redundant skin and pulmonary emphysema. Since the Marfan syndrome and congenital contractural arachnodactyly are due to mutations in different genes, it has been uncertain whether neonatal Marfan syndrome is due to mutations in the fibrillin gene on chromosome 15 or in another gene. We studied an infant with severe neonatal Marfan syndrome. Dermal fibroblasts were metabolically labeled and found to secret fibrillin inefficiently when compared with control cells. Reverse transcription and amplification of the proband's fibroblast RNA was used to identify a 3-bp insertion between nucleotides 480-481 or 481-482 of the fibrillin cDNA. The insertion maintains the reading frame of the protein and inserts a cysteine between amino acids 160 and 161 in an epidermal growth-factor-like motif of fibrillin. This 3-bp insertion was not found in the fibrillin gene in 70 unrelated, unaffected individuals and 11 unrelated individuals with the Marfan syndrome. We conclude that neonatal Marfan syndrome is the result of mutations in the fibrillin gene on chromosome 15 and is part of the Marfan syndrome spectrum. Images Figure 1 Figure 2 Figure 3 PMID:8116614

  20. Severe neonatal marfan syndrome resulting from a De Novo 3-bp insertion into the fibrillin gene on chromosome 15

    Energy Technology Data Exchange (ETDEWEB)

    Milewicz, D.M.; Duvic, M. (Univ. of Texas Medical School, Houston, TX (United States))

    1994-03-01

    Severe neonatal Marfan syndrome has features of the Marfan syndrome and congenital contractural arachnodactyly present at birth, along with unique features such as loose, redundant skin and pulmonary emphysema. Since the Marfan syndrome and congenital contractural arachnodactyly are due to mutations in different genes, it has been uncertain whether neonatal Marfan syndrome is due to mutations in the fibrillin gene on chromosome 15 or in another gene. The authors studied an infant with severe neonatal Marfan syndrome. Dermal fibroblasts were metabolically labeled and found to secrete fibrillin inefficiently when compared with control cells. Reverse transcription and amplification of the proband's fibroblast RNA was used to identify a 3-bp insertion between nucleotides 480-481 or 481-482 of the fibrillin cDNA. The insertion maintains the reading frame of the protein and inserts a cysteine between amino acids 160 and 161 in an epidermal growth-factor-like motif of fibrillin. This 3-bp insertion was not found in the fibrillin gene in 70 unrelated, unaffected individuals and 11 unrelated individuals with the Maran syndrome. The authors conclude that neonatal Marfan syndrome is the result of mutations in the fibrillin gene on chromosome 15 and is part of the Marfan syndrome spectrum. 32 refs., 3 figs.

  1. Surgical treatment of primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Brasso, K; Karstrup, S; Lundby, C M

    1994-01-01

    One hundred and two patients with primary hyperparathyroidism underwent a total of 108 bilateral neck explorations with attempted identification and biopsy of all four glands. Hypercalcaemia was surgically eliminated in 97 of 102 patients (95%). Of the remaining hypercalcaemic patients one was cu......--including bilateral neck exploration and attempted biopsies of all parathyroid glands--is safe with a high cure rate....

  2. Osteoprotegerin Levels in Primary Hyperparathyroidism

    DEFF Research Database (Denmark)

    Stilgren, L S; Hegedüs, L M; Beck-Nielsen, H

    2003-01-01

    The effect of parathyroid hormone (PTH) on the production of osteoprotegerin (OPG) remains controversial. Most in vitro studies indicate that PTH decreases OPG secretion by the osteoblast, but in vivo observations are conflicting. In primary hyperparathyroidism (PHPT), hypersecretion of PTH leads...

  3. Primary hyperparathyroidism: adenoma or hyperplasia

    OpenAIRE

    2012-01-01

    Introduction: primary hyperparathyroidism (PH) is a symptomatic or asymptomaticclinical entity, characterized by the autonomous production of parathyroid hormone (PTH). Its classical presentation exhibits an increase of the serum calcium andparathyroid hormone levels. Parathyroid scintigrafy, among other imaging modalities,is frequently used to detect the hyperfunctioning glands.Clinical case: number 1. Female, 69 years old, asymptomatic, hypertension of recentonset, osteopenia, increased int...

  4. Genetics Home Reference: hyperparathyroidism-jaw tumor syndrome

    Science.gov (United States)

    ... Genetics Home Health Conditions hyperparathyroidism-jaw tumor syndrome hyperparathyroidism-jaw tumor syndrome Enable Javascript to view the ... boxes. Download PDF Open All Close All Description Hyperparathyroidism-jaw tumor syndrome is a condition characterized by ...

  5. [Etiology and pathogenesis of primary hyperparathyroidism.

    Science.gov (United States)

    Yamauchi, Mika; Sugimoto, Toshitsugu

    2017-01-01

    Primary hyperparathyroidism(pHPT)is a frequent endocrine disease in which abnormal calcium(Ca)regulation leads to hypercalcemia. The most frequent cause of pHPT in more than 80% of patients is an adenoma, followed by hyperplasia in about 15%, and cancer in 1~5%. Although most cases of pHPT are sporadic, a few are familial(hereditary), and this is known as familial hyperparathyroidism(FHPT). Gene abnormalities that affect cyclin D1 signaling(CCND1, CDC73, CDKN1B), Wnt/β-catenin signaling(MEN1), and calcium-sensing receptor signaling(CaSR, GNA11, AP2S1)play a role in the etiology and pathogenesis of pHPT. Vitamin D insufficiency/deficiency and CaSR dysfunction also play a role in pHPT severity. Continued elucidation of the etiology and pathogenesis of pHPT may lead to development of new treatments for pHPT as well as further understanding of Ca regulation.

  6. A novel epsilon gamma delta beta thalassemia presenting with pregnancy complications and severe neonatal anemia.

    Science.gov (United States)

    Shalev, Hanna; Landau, Daniela; Pissard, Serge; Krasnov, Tanya; Kapelushnik, Joseph; Gilad, Oded; Broides, Arnon; Dgany, Orly; Tamary, Hannah

    2013-02-01

    The epsilon gamma delta beta (εγδβ)-thalassemias are rare sporadic disorders caused by deletion of the β-globin gene cluster. The main clinical feature is marked prenatal and neonatal anemia that resolves spontaneously within a few months. Reports originating mainly from Europe have so far identified 30 such deletions The aim of the present work was to describe a novel 1.78-Mb deletion, the longest ever reported, and to detail the clinical features in 12 members of an extended Bedouin family. The deletion was identified by globin gene multiplex ligation-dependent probe amplification (MLPA) of the β-globin cluster and further characterized by comparative genomic hybridization. Past and present clinical and laboratory data of ten symptomatic and two asymptomatic patients were collected. A 1.78-Mb εγδβ-deletion, the largest ever described, was identified in all patients. Although other genes were included in the deletion, no other symptoms were observed. Of the ten symptomatic fetuses and neonates, three died of the disease. The remainder required packed cell transfusions during the first months of life. Pregnancy complications included intrauterine growth restriction and oligohydramnios, as well as additional neonatal complications including prematurity and persistent pulmonary hypertension of the neonate. We suggest that εγδβ-thalassemia be added to the list of hemoglobinopathies that can cause neonatal anemia and that MLPA of the β-globin cluster be used to confirm its diagnosis. Careful surveillance during pregnancy is important to reduce neonatal mortality and morbidity, especially given the dramatic improvement that occurs later. © 2012 John Wiley & Sons A/S.

  7. Novel α-spectrin mutation in trans with α-spectrin causing severe neonatal jaundice from hereditary spherocytosis.

    Science.gov (United States)

    Nussenzveig, Roberto H; Christensen, Robert D; Prchal, Josef T; Yaish, Hassan M; Agarwal, Archana M

    2014-01-01

    We evaluated a neonate with severe jaundice but a negative family history. Spherocytes were present and suspected hereditary spherocytosis was confirmed by osmotic fragility and eosin-5-maleimide erythrocyte staining. We found he was a compound heterozygote for two pathogenic mutations in the gene encoding α-spectrin: a previously reported α(LEPRA) inherited from his asymptomatic mother, and a novel α-spectrin mutation in intron 45 +1 disrupting the consensus splice site, from his asymptomatic father.

  8. Unusual florid skeletal manifestations of primary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Ashebu, Samuel D.; Dahniya, Mohamed H.; Muhtaseb, Sayeed A.; Aduh, Prosper [Department of Radiology, Al-Adan Hospital (Kuwait)

    2002-12-01

    We report a case of primary hyperparathyroidism (PHPT) with advanced and unusual skeletal manifestations - a rare occurrence in developed countries nowadays. The literature is briefly reviewed. (orig.)

  9. Oral Manifestations of Chronic Kidney Disease and Renal Secondary Hyperparathyroidism: A Comparative Review.

    Science.gov (United States)

    Davis, Eric M

    2015-01-01

    Recent epidemiological studies have demonstrated that significant associations exist between oral disease and diseases involving non-oral tissues. Occasionally, the roles may be reversed and the oral cavity can be severely affected by systemic disease originating in another part of the body. Renal secondary hyperparathyroidism is a common endocrinopathy that occurs as a consequence of chronic azotemic kidney disease. Renal osteodystrophy, the most dramatic clinical consequence of renal secondary hyperparathyroidism is uncommon, but can result in demineralization of maxillofacial bones, loosening of teeth, and pathological jaw fractures. The purpose of this report is to update the current understanding of the pathophysiology of this endocrine disease and to compare the oral manifestations of renal secondary hyperparathyroidism in humans and companion animals. A 50-year review of the veterinary literature was undertaken to examine the clinical presentation of renal osteodystrophy in dogs, and to determine what clinical consequences of renal secondary hyperparathyroidism have been reported in domestic cats.

  10. Primary hyperparathyroidism in children and adolescents.

    Science.gov (United States)

    George, Joe; Acharya, Shrikrishna V; Bandgar, Tushar R; Menon, Padma S; Shah, Nalini S

    2010-02-01

    Primary hyperparathyroidism (PHPT) in children and adolescents is a rare condition. Awareness should improve in order to lower threshold for screening and allow intervention before serious and permanent sequelac occur. A retrospective analysis of 15 children and adolescents with PHPT (age multiple endocrine neoplasia (MEN). After biochemical confirmation, tumor was localised in all prior to surgery. Histopathology confirmed adenoma in all cases. Post-operative hungry bone syndrome occurred in 33.3%. PHPT is more common in females. Presentation of the disease is similar to their adult counterparts except for more severe bone disease and less severe renal disease. MEN and familial non-MEN PHPT do not constitute a major cause of pediatric PHPT as against to worldwide data. The incidence of hyperplasia as a cause of PHPT is rare in our pediatric population.

  11. Partial response to cinacalcet treatment in a patient with secondary hyperparathyroidism undergoing hemodialysis: a case report

    Science.gov (United States)

    2012-01-01

    Introduction In the treatment of secondary hyperparathyroidism of chronic kidney disease, calcimimetics - allosteric modulators of the calcium-sensing receptor - inhibit glandular hyperplasia and significantly reduce circulating parathyroid hormone levels. They have a major impact on the management of secondary hyperparathyroidism. Case presentation We present the clinical case of a 41-year-old Caucasian man undergoing chronic hemodialysis, who had a parathyroidectomy to treat severe secondary hyperparathyroidism resistant to cinacalcet treatment. Preoperatively, 24 months after high-dose cinacalcet hydrochloride, we observed a persistently elevated intact parathyroid hormone serum level, and detected clear parathyroid gland hyperplasia regression on ultrasound. We performed a three-gland parathyroidectomy, which was assumed to be total, associated with a hemithyroidectomy. Our patient then entered a hypoparathyroid state. A histopathological examination showed that the removed parathyroid glands were of small size, with a total weight of 1g, associated with a multifocal small papillary thyroid cancer. Conclusion In the management of secondary hyperparathyroidism, cinacalcet hydrochloride effectively reduces total parathyroid gland hyperplasia. However, a persisting elevated intact parathyroid hormone serum level may be observed, demonstrating that reduced parathyroid hyperplastic tissue may still be associated with severe secondary hyperparathyroidism. Even if calcimimetics are very effective in secondary hyperparathyroidism treatment, further studies are necessary for a better understanding of their actions. PMID:23232027

  12. Partial response to cinacalcet treatment in a patient with secondary hyperparathyroidism undergoing hemodialysis: a case report

    Directory of Open Access Journals (Sweden)

    Conzo Giovanni

    2012-12-01

    Full Text Available Abstract Introduction In the treatment of secondary hyperparathyroidism of chronic kidney disease, calcimimetics - allosteric modulators of the calcium-sensing receptor - inhibit glandular hyperplasia and significantly reduce circulating parathyroid hormone levels. They have a major impact on the management of secondary hyperparathyroidism. Case presentation We present the clinical case of a 41-year-old Caucasian man undergoing chronic hemodialysis, who had a parathyroidectomy to treat severe secondary hyperparathyroidism resistant to cinacalcet treatment. Preoperatively, 24 months after high-dose cinacalcet hydrochloride, we observed a persistently elevated intact parathyroid hormone serum level, and detected clear parathyroid gland hyperplasia regression on ultrasound. We performed a three-gland parathyroidectomy, which was assumed to be total, associated with a hemithyroidectomy. Our patient then entered a hypoparathyroid state. A histopathological examination showed that the removed parathyroid glands were of small size, with a total weight of 1g, associated with a multifocal small papillary thyroid cancer. Conclusion In the management of secondary hyperparathyroidism, cinacalcet hydrochloride effectively reduces total parathyroid gland hyperplasia. However, a persisting elevated intact parathyroid hormone serum level may be observed, demonstrating that reduced parathyroid hyperplastic tissue may still be associated with severe secondary hyperparathyroidism. Even if calcimimetics are very effective in secondary hyperparathyroidism treatment, further studies are necessary for a better understanding of their actions.

  13. DIFFERENTIAL DIAGNOSIS OF NORMOCALCEMIC HYPERPARATHYROIDISM

    Directory of Open Access Journals (Sweden)

    N. A. Kravchun

    2015-01-01

    Full Text Available Objective: to describe a case of normocalcemic hyperparathyroidism.Materials and methods. A female patient aged 51 years sought medical advice for complaints of spinal column and bone pains, periodic dizziness, and hand numbness. The patient underwent clinical and biochemical blood tests, determination of the blood levels of calcium, phosphorus, parathyroid hormone, concentrations of total vitamin D, calciuria, electrocardiography, thyroid ultrasonography, and neck computed tomography.Results. Based on her complaints, examination evidence, evaluation of the clinical presentations of the disease, and objective and instrumental examination findings, the patient was diagnosed with left parathyroid adenoma and primary hyperparathyroidism; stage II hypertensive disease, grade I, a moderate risk; retinal angiopathy of both eyes; stage I dyscirculatory encephalopathy with liquor and venous dyscirculation and moderate vestibular ataxia; urolithiasis; kidney stones; and spondylosis mainly involving the lumbar spine. The diagnostic determinants for verifying the diagnosis were the results of neck computed tomography, namely: the signs of space-occupying lesion in the projection of the left parathyroid gland; the blood level of parathyroid hormone 118.6 pg/ml (normal value (N 9.5–75.0 pg/ml; total vitamin D 21.64 nmol/l (N 75–250 nmol/l; and calcium 2.48 mmol/l (N 2.15–2.50 mmol/l. The history of urolithiasis, repeated lithotripsy, as well as spondylosis with lumboischalgia was an absolute indication for surgical treatment.Conclusion. The practical interest in this case is due to the complexity of diagnosing normocalcemic hyperparathyroidism after vitamin D deficiency. The early stages of primary hyperparathyroidism are asymptomatic and frequently give rise to irreversible renal complications, causing renal failure and, as a consequence, disability. This clinical case demonstrates the importance of routinely determining vitamin D concentrations

  14. Risk Factors Affecting the Severity of Full-Term Neonatal Retinal Hemorrhage

    Directory of Open Access Journals (Sweden)

    Zhang Yanli

    2017-01-01

    Full Text Available Objective. The purpose of this study was to explore the underlying clinical factors associated with the degree of retinal hemorrhage (RH in full-term newborns. Methods. A total of 3054 full-term infants were included in this study. Eye examinations were performed with RetCamIII within one week of birth for all infants. Maternal, obstetric, and neonatal parameters were compared between newborns with RH and controls. The RH group was divided into three sections (I, II, and III based on the degree of RH. Results. RH was observed in 1202 of 3054 infants (39.36% in this study. The quantity and proportion of newborns in groups I, II, and III were 408 (13.36%, 610 (19.97%, and 184 (6.03%, respectively. Spontaneous vaginal delivery (SVD, prolonged duration of second stage of labor, advanced maternal age, and neonatal intracranial hemorrhage positively correlated with aggravation of the degree of RH in newborns. Conversely, cesarean section was protective against the incidence of RH. Conclusions. SVD, prolonged duration of second stage of labor, advanced maternal age, and neonatal intracranial hemorrhage were potential risk factors for aggravation of the degree of RH in full-term infants. Accordingly, infants with these risk factors may require greater attention with respect to RH development.

  15. [Oral calcimimetics on secondary hyperparathyroidism of uremia.

    Science.gov (United States)

    Imanishi, Yasuo

    2017-01-01

    Secondary hyperparathyroidism of uremia is associated with poor health outcomes, including all-cause mortality, cardiovascular mortality, and fractures. Standard therapy for secondary hyperparathyroidism includes vitamin D receptor activators, and phosphate binders. Persistently elevated parathyroid hormone(PTH)levels may require the addition of calcimimetics which sensitizes calcium-sensing receptors on the parathyroid glands.

  16. Dominantly inherited isolated hyperparathyroidism: a syndromic association?

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K. [Department of Radiology, Royal Alexandra Hospital for Children, Sydney (Australia)]|[Department of Radiology, New Children`s Hospital, PO Box 3515, Parramatta, NSW 2124 (Australia); Czerminska-Kowalska, A. [Department of Radiology, Children`s Memorial Health Institute, Warsaw (Poland); Kulczycka, H.; Rowinska, E.; Pronicka, E. [Department of Metabolism, Children`s Memorial Health Institute, Warsaw (Poland)

    1999-01-01

    Dominantly inherited isolated hyperparathyroidism (DIIH) is rare in childhood. It may be the first biochemical abnormality in the multiple endocrine neoplasia type I (MEN I) and type II (MEN II) syndromes. Its clinical course is usually asymptomatic or of low morbidity. Radiographic examination is most often normal. We describe six members of a family with distinctive phenotype and DIIH. Limited systemic symptoms and severe radiographic osteitis fibrosa cystica were further unusual features in this family. The diagnosis of DIIH was made only after a 9-year-old girl developed hypercalcaemic crisis after a pathological femoral fracture. Distinctive phenotype, unusual clinical course and unparalleled radiographic changes suggest a not yet described syndromic association. (orig.) With 7 figs., 3 tabs., 23 refs.

  17. Edaravone, a hydroxyl radical scavenger, ameliorates the severity of pulmonary hypertension in a porcine model of neonatal sepsis.

    Science.gov (United States)

    Yamaguchi, Sachiko; Hussein, Mohamed Hamed; Daoud, Ghada AbdEl-Hamid; Goto, Tatenobu; Kato, Shin; Kakita, Hiroki; Mizuno, Haruo; Ito, Tetsuya; Fukuda, Sumio; Kato, Ineko; Suzuki, Satoshi; Hashimoto, Takashi; Togari, Hajime

    2011-01-01

    Systemic infection in the newborn (neonatal sepsis) is the most common cause of neonatal mortality. Neonatal sepsis is complicated by pulmonary hypertension. In this study, we analyzed the effect of edaravone, a free radical scavenger that is known to reduce the production of inflammatory mediators, such as tumor necrosis factor α (TNFα), on pulmonary hypertension. Experimental and sham groups were drawn from 19 three-day-old piglets; 5 underwent a modified procedure of cecal ligation and perforation (CLP) (CLP group), 8 underwent CLP followed 30 min later by edaravone intravenous administration (edaravone group), and 6 did not undergo CLP and did not receive edaravone (sham group). To evaluate the pulmonary blood pressure despite the sepsis-induced low cardiac output, mean arterial blood pressure (mABP), mean pulmonary arterial pressure (mPAP), and comparative pulmonary hypertension ratio (mPAP/mABP) were determined. Serum TNFα levels were measured before the procedure and at 1, 3, and 6 h after. The mPAP levels were higher in the CLP group at 9 h compared to the edaravone group. The mPAP/mABP ratio was lower in the edaravone and sham groups compared to the CLP group at 6 and 9 h. TNFα in the edaravone and sham groups were lower at 1 and 3 h compared to that in the CLP group. In all animals, mPAP/mABP at 6 h correlated with serum levels of TNFα at 1, 3, and 6 h. These findings suggest that edaravone ameliorates the severity of pulmonary hypertension in a neonatal sepsis model by reducing serum TNFα levels.

  18. Acute necrotizing pancreatitis as fi rst manifestation of primary hyperparathyroidism

    Institute of Scientific and Technical Information of China (English)

    Jeroen; I; Lenz; Jimmy; M; Jacobs; Bart; Op; de; Beeck; Ivan; A; Huyghe; Paul; A; Pelckmans; Tom; G; Moreels

    2010-01-01

    We report the case of a female patient with severe acute necrotizing pancreatitis associated with hypercalcemia as first manifestation of primary hyperparathyroidism caused by a benign parathyroid adenoma.Initially the acute pancreatitis was treated conservatively.The patient subsequently underwent surgical resection of the parathyroid adenoma and surgical clearance of a large infected pancreatic pseudocyst.Although the association of parathyroid adenoma-induced hypercalcemia and acute pancreatitis is a kno...

  19. Tumoral calcinosis, calciphylaxis, hyperparathyroidism and tuberculosis in a dialysis patient

    Directory of Open Access Journals (Sweden)

    Khawla Kammoun

    2011-01-01

    Full Text Available Tumoral calcinosis and calciphylaxis are uncommon but severe complications in ure-mic patients. They occur generally after long-term hemodialysis (HD treatment explained by ad-vanced secondary hyperparathyroidism and longstanding high calcium phosphorus product (Ca × P. Other factors such granulomatous diseases may worsen the calcium phosphate homeostasis alterations. We report a young male patient treated by HD for 6 years who developed tuberculosis in addition to tumoral calcinosis and calciphylaxis.

  20. Calcification of all four parathyroid glands in a hemodialysis patient with secondary hyperparathyroidism revealed by computerized tomography.

    Science.gov (United States)

    Peces, R; Rodríguez, M; González, F; Ablanedo, P

    2001-09-01

    This report describes the parathyroid scan, computerized tomography and histologic findings in a young female hemodialysis patient with severe secondary hyperparathyroidism. These findings included hyperplasia and calcification of all four parathyroid glands.

  1. Brown tumor of secondary hyperparathyroidism: surgical approach and clinical outcome.

    Science.gov (United States)

    Queiroz, Isaac Vieira; Queiroz, Samara Pereira; Medeiros, Rui; Ribeiro, Rodolfo Bonfim; Crusoé-Rebello, Iêda Margarida; Leão, Jair Carneiro

    2016-12-01

    Secondary hyperparathyroidism is a frequent complication of chronic renal failure. The brown tumor is an unusual presentation of fibrous osteitis that represents a serious complication of renal osteodystrophy, affecting predominantly the hands, feet, skull, and facial bones. The aim of this paper is to describe the case of a 53-year-old female patient, with renal failure who has been on dialysis for 6 years and developed severe secondary hyperparathyroidism and brown tumor of the maxilla and mandible, confirmed by incisional biopsy. Parathyroidectomy was indicated as a result of rapid growth of the tumor and the maintenance of laboratory findings. Despite the normalization of serum parathyroid hormone and alkaline phosphatase, tumor regression was slow and patient's important functional and esthetic deficits persisted. Excision of the mandible tumor was conservative. Osteoplasty was recommended because during a 5-year follow-up there was regression of the lesion, decreased pain, bleeding, and tooth mobility.

  2. Preoperative localization in primary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Han, Sanson; Kitamura, Hiroyuki; Takagita, Shin-ichi; Maetani, Toshiki; Iwahashi, Yuka; Miyazaki, Masakazu; Yamamoto, Norio [Tenri Hospital, Nara (Japan)

    1998-04-01

    Between 1992 and 1996, 31 cases (8 men and 23 women) with primary hyperparathyroidism (PHPT) were treated in our department. In this study, we compared which of the preoperative localization methods was most useful in the detection of PHPT. The sensitivity for detection of abnormal parathyroid glands was 88.6% on ultrasonography (US), 76.9% on magnetic resonance imaging (MRI), 74.3% on Tl-Tc subtraction scintigraphy and 68.8% on computed tomography. We concluded that US should be performed first, with MRI as a supplementary method, for the detection of abnormal parathyroid glands and the evaluation of invasion within the body. (author)

  3. Sick sinus syndrome secondary to primary hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Osman Beton

    2016-03-01

    Full Text Available The effects of hypercalcemia on the heart and the resulting alternations of the electrocardiogram have been well established. However, there are only limited number of reports in the literature on primary hyperparathyroidism leading to clinically significant arrhythmias. We present a patient who was diagnosed with symptomatic sick sinus syndrome in the setting of moderate hyper-calcemia secondary to primary hyperparathyroidism in this case report. After the surgical opera-tion for primary hyperparathyroidism, serum calcium level returned to normal range and patient’s complaints and arrhytmic findings recovered. The patient was asymptomatic for the following 13 years.

  4. Myofibroblastic tumor causing severe neonatal distress. Successful surgical resection after embolization.

    Science.gov (United States)

    Castañón, Montserrat; Saura, Laura; Weller, Santiago; Prat, Jordi; Thio, Marta; Sorolla, Juan P; Albert, Asteria; Morales, Lluís

    2005-06-01

    This report describes a case of a term male 3.1 kg, normal delivery, 38 weeks of gestation with a record of hydramnios by prenatal sonography. He had fetal acute suffering and respiratory distress. The first radiographic study showed a mass filling the whole left thorax cage causing erosion of the inferior edge of the third rib. The mediastinum was displaced to the right. Computed tomography scan confirmed a homogeneous tumor that filled the left thorax and displaced the mediastinum to the right without invasion. Surgical biopsy informed of a highly vascularized mesenchymal tumor. The tumor was embolized with Ivalon microparticles obtaining a nearly avascular mass. Complete surgical excision was made, including the whole mass and costal segments. Microscopically, it was an inflammatory myofibroblastic tumor. It was composed mainly of spindle-shaped cells without malignant features. On immunohistochemistry, the tumor showed positive staining for vimentin, whereas antidesmin antibodies and S-100 protein were negative. The aim of this article is to present an extremely uncommon case of neonatal distress caused by an intrathoracic, extrapulmonary myofibroblastic tumor. Complete surgical resection was possible after embolization.

  5. Parathyroid scintigraphy during hypocalcaemia in primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Øgard, Christina G; Thomsen, Jørn Bo; Jakobsen, Henrik

    2005-01-01

    Minimally invasive parathyroid surgery in patients with primary hyperparathyroidism (PHPT) demands high imaging accuracy. By increasing blood flow to the parathyroid adenoma before injection of a perfusion marker, we intended to improve the parathyroid scintigraphy. We have named the technique...

  6. The Surgical Management of Primary Hyperparathyroidism: The ...

    African Journals Online (AJOL)

    The Surgical Management of Primary Hyperparathyroidism: The Experience in ... the gastrointestinal system in the form of dyspepsia from Peptic ulcer disease and ... years from 2007-2014 and only three had complete medical documents.

  7. Hyperparathyroidism: molecular, diagnostic and therapeutic aspec

    Directory of Open Access Journals (Sweden)

    Mikołaj Pietkiewicz

    2010-10-01

    Full Text Available The sensitivity of parathyroid glands to a low calcium level in plasma results in parathyroid hormone (PTH release in order to restore the normal Ca2 concentration. Hyperparathyroidism is a common endocrinopathy, caused by uncontrolled growth of parathyroid cells. In primary hyperparathyroidism, hypercalcemia develops due to extensive autonomous secretion of PTH. Secondary hyperparathyroidism is a well-established complication of chronic renal insufficiency, where marked parathyroid hyperplasia occurs, especially in patients with long dialysis vintage. The elevated PTH level in the circulation is a direct result of renal function disturbances, vitamin D deficiency, and impaired calcium/phosphate metabolism. After successful kidney transplantation, the normalization of kidney function fails to normalize the secretion of PTH by parathyroid glands, which have become relatively autonomous and unresponsive to hypercalcemic conditions in the plasma. The development of tertiary hyperparathyroidism occurs in these conditions.The aim of our report is to present current views on the clinical, pathological and biochemical features of primary, secondary and tertiary hyperparathyroidism. The diagnostics of calcium/phosphate abnormalities in parathyroid gland disorders, as well as some aspects of hyperparathyroidism treatment, are briefly summarized.

  8. Hyperparathyroidism caused by distant pulmonary lesions and parathyromatosis after ethanol injection/parathyroidectomy for secondary hyperparathyroidism.

    Science.gov (United States)

    Nakamura, Michio; Tanaka, Kiho; Fujii, Takeshi

    2017-01-11

    Secondary hyperparathyroidism (SHPT) treatment includes parathyroidectomy and percutaneous ethanol injection therapy (PEIT), which are invasive procedures. The condition in which benign hyperfunctioning parathyroid tissue is distributed throughout the neck and mediastinum is termed parathyromatosis. Here, we present the case of a 51-year-old woman who began hemodialysis in 1986 due to chronic kidney disease of unknown etiology and developed SHPT in 1999. She underwent 6 rounds of PEIT followed by total a parathyroidectomy with partial forearm autotransplantation. Between 2011 and 2013, surgeons removed several nodules from her pulmonary and cervical regions and the transplanted masses from her forearm; all showed hyperplasia but exhibited no histological evidence of malignancy. Damage to the parathyroid capsule after repeated PEITs may cause local cervical recurrence and pulmonary lesions, although distant lesions are extremely rare in SHPT. This case is of interest due to the possible association between PEIT and parathyromatosis and distal lesions.

  9. Risk factors for severe neonatal hyperbilirubinemia in low and middle-income countries: a systematic review and meta-analysis.

    Directory of Open Access Journals (Sweden)

    Bolajoko O Olusanya

    Full Text Available Available evidence suggests that low- and middle-income countries (LMICs bear the greatest burden of severe neonatal hyperbilirubinemia characterized by disproportionately high rates of morbidity, mortality and neurodevelopmental disorders compared to high-income countries. We set out to identify the risk factors that contribute to the burden of severe hyperbilirubinemia in the most developmentally disadvantaged LMICs to highlight areas for action and further research.We systematically searched PubMed, Scopus, Ovid EMBASE, Cumulative Index to Nursing and Allied Health Literature (CINAHL, WHO Library Database (WHOLIS, African Index Medicus (AIM, African Journals Online (AJOL, LILACS, and IndMed for reports published between January 1990 and June 2014. We included only studies that controlled for the effects of confounding variables in determining maternal and infant risk factors for severe hyperbilirubinemia. We conducted meta-analysis of the eligible studies and computed the summary risk estimates with random effects models.A total of 13 studies with 1,951 subjects and 32,208 controls from India, Nigeria, Pakistan, Nepal and Egypt were identified and analyzed. The pooled data showed that primiparity (OR, 1.59; 95% CI:1.26-2.00, delivery outside public hospitals (OR, 6.42; 95% CI:1.76-23.36, ABO incompatibility (OR, 4.01; 95% CI:2.44-6.61, Rhesus hemolytic disease (OR, 20.63; 95% CI:3.95-107.65, G6PD deficiency (OR, 8.01; 95% CI:2.09-30.69, UGT1A1 polymorphisms (OR, 4.92; 95% CI:1.30-18.62, low gestational age (OR, 1.71; 95% CI:1.40-2.11, underweight/weight loss (OR, 6.26; 95% CI:1.23-31.86, sepsis (OR, 9.15; 95% CI:2.78-30.10 and high transcutaneous/total serum bilirubin levels (OR, 1.46; 95% CI:1.10-1.92 placed infants at increased risk of severe hyperbilirubinemia or bilirubin induced neurologic dysfunctions. Low social class was not associated with an increased risk of severe hyperbilirubinemia.Infants at risk of severe hyperbilirubinemia in

  10. Multifactorial Hypercalcemia and Literature Review on Primary Hyperparathyroidism Associated with Lymphoma

    Directory of Open Access Journals (Sweden)

    Jelena Maletkovic

    2014-01-01

    Full Text Available The most common cause of hypercalcemia in hospitalized patients is malignancy. Primary hyperparathyroidism most commonly causes hypercalcemia in the outpatient setting. These two account for over 90% of all cases of hypercalcemia. Hypercalcemia can be divided into PTH-mediated and PTH-independent variants. Primary hyperparathyroidism, familial hypocalciuric hypercalcemia, familial hyperparathyroidism, and secondary hyperparathyroidism are PTH mediated. The most common PTH-independent type of hypercalcemia is malignancy related. Several mechanisms lead to hypercalcemia in malignancy-direct osteolysis by metastatic disease or, more commonly, production of humoral factors by the primary tumor also known as humoral hypercalcemia of malignancy that accounts for about 80% of malignancy-related hypercalcemia. The majority of HHM is caused by tumor-produced parathyroid hormone-related protein and less frequently production of 1,25-dihydroxyvitamin D or parathyroid hormone by the tumor. We report the rare case of a patient with hypercalcemia and diagnosed primary hyperparathyroidism. The patient had persistent hypercalcemia after surgical removal of parathyroid adenoma with recorded significant decrease in PTH level. After continued investigation it was found that the patient also had elevated 1,25-dihydroxyvitamin D and further studies confirmed a large spleen mass that was later confirmed to be a lymphoma. This is a rare example of two concomitant causes of hypercalcemia requiring therapy.

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

    Directory of Open Access Journals (Sweden)

    Flavia L. Coutinho

    2012-01-01

    Full Text Available 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.

  12. Potential utility of cinacalcet as a treatment for CDC73-related primary hyperparathyroidism: a case report.

    Science.gov (United States)

    Sato, Takeshi; Muroya, Koji; Hanakawa, Junko; Yamashita, Sumimasa; Nozawa, Kumiko; Masudo, Katsuhiko; Yamakawa, Tadashi; Asakura, Yumi; Hasegawa, Tomonobu; Adachi, Masanori

    2016-07-01

    We report a Japanese pedigree with familial primary hyperparathyroidism due to a CDC73 mutation. To our knowledge, this is the first report of cinacalcet as a treatment for CDC73-related primary hyperparathyroidism. The proband had severe psychomotor retardation and received laryngotracheal separation surgery. At 19 yr of age, he developed acute pancreatitis. Hypercalcemia (12.2-13.8 mg/dL), elevated levels of intact PTH (86-160 pg/mL), and a tumor detected upon neck ultrasonography led to the diagnosis of primary hyperparathyroidism. Family history and biochemical examinations revealed that three family members (the proband's mother, elder brother, and maternal grandfather) had primary hyperparathyroidism. We identified a novel heterozygous mutation, c.240delT, p.Glu81Lysfs*28, in the CDC73 gene in three affected family members, excluding the proband's elder brother who refused genetic testing. Parathyroidectomy for the proband was considered as high-risk, because the tumor was located close to the tracheostomy orifice. After receiving approval from the institutional review board and obtaining the consent, we initiated cinacalcet treatment. At 22 yr of age, treatment with 100 mg of cinacalcet maintained serum calcium levels below 11.0 mg/dL with no apparent side effects. Our report presents the potential efficacy of cinacalcet as a treatment for CDC73-related primary hyperparathyroidism, in particularly inoperative cases.

  13. Carnitine in neonatal nutrition.

    Science.gov (United States)

    Borum, P R

    1995-11-01

    Experimental evidence from several investigators suggests that carnitine is a conditionally essential nutrient for neonates. If carnitine is a conditionally essential nutrient for the neonate, most neonates on total parenteral nutrition in the United States are not receiving adequate nutritional support. The metabolic functions of carnitine are varied and important in several aspects of neonatal physiology. All neonates receiving breast milk receive dietary carnitine and most neonates receiving enteral infant formulas receive dietary carnitine at a level similar to that of the breast-fed neonate. However, most neonates on total parenteral nutrition receive no dietary carnitine. Investigators have been testing the working hypothesis that carnitine is a conditionally essential nutrient for the neonate for many years. This review discusses (1) data supporting the hypothesis, (2) reasons why it has not been either proved or disproved by now, and (3) the author's view of a prudent approach to dietary carnitine supplementation of neonates.

  14. Hyperparathyroidism

    Science.gov (United States)

    ... Hajj Fuleihan G. Pathogenesis and etiology of primary hyperthyroidism. http://www.uptodate.com/home. Accessed Feb. 15, 2014. Papadakis MA, et al. Current Medical Diagnosis & Treatment 2014. 53rd ed. New York, N.Y.: The ...

  15. Primary hyperparathyroidism in pregnancy leading to hypercalcaemic crisis and uraemic encephalopathy

    Science.gov (United States)

    Nash, E; Ranka, P; Tarigopula, G; Rashid, T

    2015-01-01

    Primary hyperparathyroidism is rare during pregnancy. Recognition may be challenging as symptoms overlap with those of other common disorders of pregnancy. Furthermore, physiological changes of pregnancy affecting calcium homoeostasis mean awareness of the condition and careful interpretation of results in the light of pregnancy are essential for diagnosis. Maternal complications of primary hyperparathyroidism include nephrolithiasis, pancreatitis, cardiac arrhythmias, hypertension and peptic ulcers. At its most severe, hypercalcaemic crisis may occur, presenting with acute neurological disturbance. Most commonly, the underlying aetiology is a solitary parathyroid adenoma whereby parathyroidectomy is the only cure. A 30-year-old Caucasian woman booked under Consultant care presented at 32 weeks gestation with vomiting and right-sided loin pain. Following presentation, she was diagnosed with renal calculi. She was delivered by caesarean section (CS) due to deterioration in renal function. Post-CS, she had a grand mal seizure. She was found to have hypercalcaemia with underlying hyperparathyroidism. PMID:25819820

  16. Medical treatment of primary, secondary, and tertiary hyperparathyroidism

    DEFF Research Database (Denmark)

    Thomsen, Súsanna v.; Vestergaard, Peter

    2011-01-01

    Hyperparathyroidism is a condition with elevated parathyroid hormone (PTH). The increase may be due to a) primary hyperparathyroidism which is caused by adenoma of one or more parathyroid glands or hyperplasia of all four glands, b) secondary hyperparathyroidism, which may be caused by deficiency...

  17. Clinical Significance of UGT1A1 Genetic Analysis in Chinese Neonates with Severe Hyperbilirubinemia

    Directory of Open Access Journals (Sweden)

    Hui Yang

    2016-08-01

    Conclusion: Besides G6PD-deficiency screening, UGT1A1 genetic analysis, and especially the UGT1A1*6(c.211G>A, p.Arg71Gly polymorphism detection, may be taken into consideration for early diagnosis and treatment of severe hyperbilirubinemic newborns in southern China.

  18. Primary Hyperparathyroidism [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Leonardo Bandeira

    2016-01-01

    Full Text Available Over the past several generations, primary hyperparathyroidism (PHTP has undergone a change in its clinical presentation in many countries from a symptomatic disease to an asymptomatic one. The reasons for this change in clinical presentation are related to the widespread use of biochemical screening tests, to the measurement of PTH more routinely in the evaluation of metabolic bone disease and to the status of vitamin D sufficiency in the population. Along with recognition of a broader clinical spectrum of disease, including a more recently recognized normocalcemic variant, has come an appreciation that the evaluation of classic target organs that can be affected in PHPT, such as the skeleton and the kidneys, require more advanced imaging technology for complete evaluation. It is clear that even in asymptomatic patients, evidence for microstructural disease in the skeleton and calcifications in the kidneys can be demonstrated often. Potential non-classical manifestations of PHPT related to neurocognition and the cardiovascular system continue to be of interest. As a result of these advances, revised guidelines for the management of asymptomatic PHPT have been recently published to help the clinician determine whether surgery is appropriate or whether a more conservative approach is acceptable.

  19. Secondary hyperparathyroidism disease stabilization following calcimimetic therapy.

    Science.gov (United States)

    Frazão, João; Rodriguez, Mariano

    2008-01-01

    Standard therapy for secondary hyperparathyroidism (SHPT) includes dietary calcium supplementation, active vitamin D, and phosphate binders; however, these are often insufficient to allow patients to achieve their serum parathyroid hormone (PTH), calcium and calcium-phosphorus product (Ca × P) targets. Recent preclinical studies have demonstrated that treatment with type II calcimimetics that increase the sensitivity of the calcium-sensing receptor (CaR) to calcium can reverse the alterations in CaR and vitamin D receptor expression and parathyroid cell proliferation that are associated with SHPT. These data suggest that calcimimetic treatment could stabilize disease progression and improve maintenance of treatment goals. In clinical trials involving SHPT patients, the calcimimetic cinacalcet has been shown to decrease PTH, calcium, phosphorus and Ca × P. Significant improvements were seen regardless of initial disease severity, and benefits were maintained over the course of long-term therapy (up to 4 years), indicating effective disease stabilization. In conclusion, preclinical and clinical data provide both theoretical and empirical support for the use of calcimimetics in moderate and advanced SHPT to effectively stabilize disease.

  20. Treatment with long-acting lanreotide autogel in early infancy in patients with severe neonatal hyperinsulinism.

    Science.gov (United States)

    Corda, Heike; Kummer, Sebastian; Welters, Alena; Teig, Norbert; Klee, Dirk; Mayatepek, Ertan; Meissner, Thomas

    2017-06-02

    Treatment of severe diffuse congenital hyperinsulinism (CHI) without sufficient response to diazoxide is complicated by the lack of approved drugs. Therefore, patients are often hospitalized long-term or have to undergo pancreatic surgery if episodes of severe hypoglycaemia cannot be prevented. A long-acting somatostatin analogue, octreotide, has been reported to be an effective treatment option that prevents severe hypoglycaemia in children with CHI, and its off-label use is common in CHI. However, octreotide requires continuous i.v. or s.c. infusion or multiple daily injections. Here, we report our experiences with the use of a monthly application of a long-acting somatostatin analogue, lanreotide autogel® (LAN-ATG), in early infancy. The mean blood glucose concentration within 7 days before the first LAN-ATG administration were compared to 7 days after the first LAN-ATG administration and increased by 0.75 mmol/L (range 0.39-1.19 mmol/L). In the following weeks intravenous glucose infusions, octreotide, and glucagon treatment could be successfully stopped in all patients 3-20 days after the first LAN-ATG injection without substantial worsening of the hypoglycaemia rate. Increased carbohydrate requirements could be normalized with an average reduction in the carbohydrate-intake of 7 g/kg body weight/d (range 1.75-12.8 g/kg body weight/d). Over a total of 52 treatment months, no serious adverse effects occurred. Long-term LAN-ATG treatment improved blood glucose concentrations, lowered the frequency of hypoglycaemia or allowed for normalization of oral carbohydrate intake in infants with CHI younger than 6 months of age. No severe side effects were observed. LAN-ATG might be an alternative treatment option in infants with severe CHI who lack risk factors for necrotizing enterocolitis and are not responding to current treatment regimens as an alternative to surgery after careful individual evaluation.

  1. Usefulness of diagnostic imaging in primary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Sekiyama, Kazuya; Akakura, Koichiro; Mikami, Kazuo; Mizoguchi, Ken-ichi; Tobe, Toyofusa; Nakano, Koichi; Numata, Tsutomu; Konno, Akiyoshi; Ito, Haruo [Chiba Univ. (Japan). Graduate School of Medicine

    2003-01-01

    In patients with primary hyperparathyroidism, prevention of urinary stone recurrence can be achieved by surgical removal of the enlarged parathyroid gland. To ensure the efficacy of surgery for primary hyperparathyroidism, preoperative localization of the enlarged gland is important. In the present study, usefulness of diagnostic imaging for localization of the enlarged gland was investigated in primary hyperparathyroidism. We retrospectively examined the findings of imaging studies and clinical records in 79 patients (97 glands) who underwent surgical treatment for primary hyperparathyroidism at Chiba University Hospital between 1976 and 2000. The detection rates of accurate localization were investigated for imaging techniques, such as ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI) thallium-201 and technetium-99m pertechnetate (Tl-Tc) subtraction scintigraphy and {sup 99m}Tc-methoxyisobutylisonitrile (MIBI) scintigraphy, and analysed in relation to the size and weight of the gland and pathological diagnosis. The detection rates by US, CT, MRI, Tl-Tc subtraction scintigraphy and MIBI scintigraphy were 70%, 67%, 73%, 38% and 78%, respectively. The overall detection rate changed from 50% to 88% before and after 1987. The detection rate of MIBI scintigraphy was superior to Tl-Tc subtraction scintigraphy. In primary hyperparathyroidism, improvement of accurate localization of an enlarged parathyroid gland was demonstrated along with recent advances in imaging techniques including MIBI scintigraphy. (author)

  2. RANDOMIZED EUROPEAN MULTICENTER TRIAL OF SURFACTANT REPLACEMENT THERAPY FOR SEVERE NEONATAL RESPIRATORY-DISTRESS SYNDROME - SINGLE VERSUS MULTIPLE DOSES OF CUROSURF

    NARCIS (Netherlands)

    SPEER, CP; ROBERTSON, B; CURSTEDT, T; HALLIDAY, HL; COMPAGNONE, D; GEFELLER, O; HARMS, K; HERTING, E; MCCLURE, G; REID, M; TUBMAN, R; HERIN, P; NOACK, G; KOK, J; KOPPE, J; VANSONDEREN, L; LAUFKOTTER, E; KOHLER, W; BOENISCH, H; ALBRECHT, K; HANSSLER, L; HAIM, M; OETOMO, SB; Okken, Albert; ALTFELD, PC; GRONECK, P; KACHEL, W; RELIER, JP; WALTI, H

    1992-01-01

    There is now convincing evidence that the severity of neonatal respiratory distress syndrome can be reduced by surfactant replacement therapy; however, the optimal therapeutic regimen has not been defined. This randomized European multicenter trial was designed to determine whether the beneficial ef

  3. A 2-YEAR FOLLOW-UP OF BABIES ENROLLED IN A EUROPEAN MULTICENTER TRIAL OF PORCINE SURFACTANT REPLACEMENT FOR SEVERE NEONATAL RESPIRATORY-DISTRESS SYNDROME

    NARCIS (Netherlands)

    ROBERTSON, B; CURSTEDT, T; TUBMAN, R; STRAYER, D; BERGGREN, P; KOK, J; KOPPE, J; VANSONDEREN, L; HALLIDAY, H; MCCLURE, G; REID, M; OETEMO, SB; Okken, A; SPEER, C; SCHROTER, W; SVENNINGSEN, N; WALTI, H; RELIER, JP

    1992-01-01

    The postnatal growth, respiratory status and neurodevelopmental outcome of surviving babies enrolled in the first European multicentre trial of porcine surfactant (Curosurf) replacement for severe neonatal respiratory distress syndrome, were assessed at corrected ages of 1 and 2 years. Follow up rat

  4. A Frame-Shift Mutation in CAV1 Is Associated with a Severe Neonatal Progeroid and Lipodystrophy Syndrome.

    Science.gov (United States)

    Schrauwen, Isabelle; Szelinger, Szabolcs; Siniard, Ashley L; Kurdoglu, Ahmet; Corneveaux, Jason J; Malenica, Ivana; Richholt, Ryan; Van Camp, Guy; De Both, Matt; Swaminathan, Shanker; Turk, Mari; Ramsey, Keri; Craig, David W; Narayanan, Vinodh; Huentelman, Matthew J

    2015-01-01

    A 3-year-old female patient presenting with an unknown syndrome of a neonatal progeroid appearance, lipodystrophy, pulmonary hypertension, cutis marmorata, feeding disorder and failure to thrive was investigated by whole-genome sequencing. This revealed a de novo, heterozygous, frame-shift mutation in the Caveolin1 gene (CAV1) (p.Phe160X). Mutations in CAV1, encoding the main component of the caveolae in plasma membranes, cause Berardinelli-Seip congenital lipodystrophy type 3 (BSCL). Although BSCL is recessive, heterozygous carriers either show a reduced phenotype of partial lipodystrophy, pulmonary hypertension, or no phenotype. To investigate the pathogenic mechanisms underlying this syndrome in more depth, we performed next generation RNA sequencing of peripheral blood, which showed several dysregulated pathways in the patient that might be related to the phenotypic progeroid features (apoptosis, DNA repair/replication, mitochondrial). Secondly, we found a significant down-regulation of known Cav1 interaction partners, verifying the dysfunction of CAV1. Other known progeroid genes and lipodystrophy genes were also dysregulated. Next, western blotting of lysates of cultured fibroblasts showed that the patient shows a significantly decreased expression of wild-type CAV1 protein, demonstrating a loss-of-function mutation, though her phenotype is more severe that other heterozygotes with similar mutations. This phenotypic variety could be explained by differences in genetic background. Indications for this are supported by additional rare variants we found in AGPAT2 and LPIN1 lipodystrophy genes. CAV1, AGPAT2 and LPIN1 all play an important role in triacylglycerol (TAG) biosynthesis in adipose tissue, and the defective function in different parts of this pathway, though not all to the same extend, could contribute to a more severe lipoatrophic phenotype in this patient. In conclusion, we report, for the first time, an association of CAV1 dysfunction with a syndrome

  5. Cestrum diurnum intoxication in normal and hyperparathyroid pigs.

    Science.gov (United States)

    Kasali, O B; Krook, L; Pond, W G; Wasserman, R H

    1977-04-01

    The effect of ingestion of dried leaves of Cestrum diurnum, a plant shown to contain a 1,25-dihydroxycholecalciferol-like principle, was tested in normal pigs fed 1.2% calcium and 1.0% phosphorus for 10 weeks from weaning and in hyperparathyroid pigs fed 0.8% calcium and 1.6% phosphorus for the same periods of time. Addition of 3% Cestrum diurnum leaf meal rapidly resulted in decreased feed consumption and weight gain, hypercalcemia and hypophosphatasemia. In normal pigs, plasma calcium rose to 16 mg/100 ml within one week and remained high for the 4 week experimental period. In hyperparathyroid pigs with hypocalcemia, plasma calcium rose to 12.75 mg/100 ml within one week and later approached 15 mg/100 ml. Ingestion of Cestrum diurnum retarded cell differentiation of growth cartilages. Arrested osteocytic osteolysis was observed within one week with osteopetrosis of epiphyses and metaphyses. The negative effect on the resorbing osteocytes then caused osteonecrosis which, in combination with lack of bone formation because of atrophy of osteoblasts, resulted in osteopenia within 4 weeks. Dystrophic calcinosis occurred within 2 weeks and was widespread after 4 weeks in lungs, kidneys, heart and vessels. Atrophy of parathyroid cells was severe after one week. Hyperparathyroid pigs responded with skeletal lesions, dystrophic calcinosis and parathyroid atrophy more rapidly and severely than normal pigs. The biochemical and anatomical changes in Cestrum diurnum ingestion are closely similar to those in vitamin D3 intoxication in pigs. Whereas pigs can tolerate large amounts of vitamin D3 because of feed-back control of 1 alpha-hydroxylation in the kidney, this control point is by-passed in Cestrum diurnum ingestion and intoxication occurs promptly.

  6. Leontiasis ossea in a patient with hyperparathyroidism secondary to chronic renal failure

    Energy Technology Data Exchange (ETDEWEB)

    Aggunlu, Levent; Akpek, Sergin; Coskun, Bilgen [Department of Radiology, School of Medicine, Gazi University, Besevler, Ankara (Turkey)

    2004-08-01

    Osteitis fibrosa describes the bone changes seen in renal osteodystrophy secondary to longstanding hyperparathyroidism. We report a 19-year-old man with longstanding chronic renal failure with a severe form of osteitis fibrosa affecting the jaws and other maxillofacial bones causing bizarre facial and dental deformity in a patient-uraemic leontiasis ossea. (orig.)

  7. Diagnostic Criteria and Evaluation of Severe Combined Immunodeficiency in the Neonate.

    Science.gov (United States)

    Diamond, Carrie E; Sanchez, Matthew J; LaBelle, James L

    2015-07-01

    Severe combined immunodeficiency disorders (SCID) are a group of primary immunodeficiencies resulting from any one of a diverse group of mutations impacting T-cell development. SCID is diagnosed and classified through assessment of the lymphocyte subset(s) affected and by the mechanisms responsible for the primary immune defect. Regardless of the genetics involved, patients invariably succumb to an early death without medical intervention. In the past, patients were primarily identified either by previous family history, physical manifestations, or after the onset of symptoms. However, the introduction of newborn screening for SCID has allowed the pediatrician to identify these patients at a much earlier age, greatly improving their survival. Currently, 23 states include SCID testing for T-cell deficiencies in their newborn screening platform. Protocols for confirmatory testing and medical intervention after a positive screen vary slightly from state-to-state. However, the standard curative treatment remains stem cell transplantation, although depending on the genetic cause of the disease, enzyme replacement and gene therapy may also be considered.

  8. Primary hyperparathyroidism presenting as acute gallstone pancreatitis

    Institute of Scientific and Technical Information of China (English)

    HE Jian-hong; ZHANG Quan-bao; LI Yu-min; ZHU You-quan; LI Xun; SHI Bin

    2010-01-01

    @@ Primary hyperparathyroidism (PHPT) is a generalized disorder of calcium metabolism resulting from an abnormally high level of serum calcium and an increased level of parathormone (PTH).1,2 Traditionally, symptomatic PHPT patients present with a variety of disorders including fatigue, amyotrophy, memory impairment, emotional instability, hallucination, irritation, loss of consciousness, abdominal distention, nausea, vomiting, constipation, acute pancreatitis, refractory peptic ulcer, osteopenia, hypertension, etc.3-5 Acute pancreatitis (AP) is an uncommon presentation of PHPT.6-8 We report a typical case of primary hyperparathyroidism with hypercalcemic crisis presenting as acute gallstone pancreatitis.

  9. Temporal similarity between Candida albicans genotypes in a Tunisian neonatal intensive care unit suggests several nosocomial cross-contamination episodes.

    Science.gov (United States)

    Ben Abdeljelil, Jihene; Saghrouni, Fatma; Cabaret, Odile; Boukadida, Jalel; Bretagne, Stéphane; Ben Saïd, Moncef

    2012-07-01

    The nosocomial transmission of Candida albicans in neonatal intensive care units (NICUs) is an increasing concern and understanding the route of this transmission is critical for adequate infection control measures. The aim of our study was to assess the likeliness of nosocomial acquisition of C. albicans in the NICU of Farhat Hached hospital in Sousse (Tunisia). We genotyped 82 isolates from 40 neonates and 7 isolates from 5 health care workers (HCWs) with onychomycosis, by using CDC3 microsatellite length polymorphism (MLP) and the high-resolution melting (HRM) analysis. Combined MLP and HRM CD3 analysis led to the delineation of 12 genotypes. Five temporal clustering caused by five genotypes occurred during the study period. Three of these genotypes were isolated in both neonates and HCWs. The first clustering included 28 isolates obtained between January 2003 and May 2004 from 16 neonates and 2 HCWs. The second clustering included three isolates collected in 2004 from three neonates and two HCWs. The third clustering included 11 isolates obtained from 6 neonates and 1 HCW in 2006. The two remaining clustering could not be associated with any HCW's contamination. These results argue for the nosocomial transmission of C. albicans in our NICU. The combined MLP and HRM analysis is a rapid first approach for tracking cross-contamination.

  10. Clinical observation of pulmonary function in patients of be severe secondary hyperparathyroidism caused by uremia%尿毒症继发重症甲状旁腺功能亢进症患者肺功能特征分析

    Institute of Scientific and Technical Information of China (English)

    宋玉环; 王艺萍; 覃莲香; 赵娜; 肖跃飞; 张凌; 姚力

    2014-01-01

    Objectives To evaluate the lung function in patients of severe secondary hyperparathyroidism caused by uremia and to analyze related factors.Methods The pulmonary function of 70 patients with uremia ( 40 patients with severe secondary hyperparathyroidism, 30 MHD patients without SHPT) and 30 healthy people was determined.Correlative analysis was performed between parameters such as pulmonary function indexes and clinical or laboratory parameters. Results Lung function was significantly different between patients with uremia and healthy subjects(P<0.05, respectively). A number of lung function indicators were negatively correlated with iPTH,C reactive protein( CRP) ,dialysis age but were positively correlated with hemoglobin (Hb) and albumin(Alb) (P <0.05).Conclusion Patients with SHPT show impaired lung function.IPTH, dialysis age, Hb, Alb and CRP can be used as important indicators of lung function.%目的:研究尿毒症继发重症甲状旁腺功能亢进症( secondary hyperparathyroidism,SHPT)患者肺功能损害特征及其可能的相关因素。方法应用肺功能仪检测40例重症SHPT患者的肺功能,30例同期不伴有严重SHPT的维持性血透( maintenance hemodialysis, MHD)患者及30例健康体检者作为对照组,并就SHPT组肺功能指标与全段甲状旁腺激素( intact parathyroid hormone, iPTH)等临床检验指标做相关性分析。结果 SHPT组和MHD组较健康体检者、SHPT组较MHD组肺功能均明显下降(P<0.05)。 SHPT组患者肺功能多项指标与血iPTH、C反应蛋白(C reactive protein,CRP)、透析龄(dialysis age)呈负相关(P<0.05),与血红蛋白(hemoglobin, Hb)、白蛋白(albu-min, Alb)呈正相关(P<0.05)。结论重症SHPT患者肺功能明显异常。血iPTH、透析龄、血红蛋白、白蛋白及CRP可能是影响SHPT患者肺功能的重要因素。

  11. A Frame-Shift Mutation in CAV1 Is Associated with a Severe Neonatal Progeroid and Lipodystrophy Syndrome.

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    Isabelle Schrauwen

    Full Text Available A 3-year-old female patient presenting with an unknown syndrome of a neonatal progeroid appearance, lipodystrophy, pulmonary hypertension, cutis marmorata, feeding disorder and failure to thrive was investigated by whole-genome sequencing. This revealed a de novo, heterozygous, frame-shift mutation in the Caveolin1 gene (CAV1 (p.Phe160X. Mutations in CAV1, encoding the main component of the caveolae in plasma membranes, cause Berardinelli-Seip congenital lipodystrophy type 3 (BSCL. Although BSCL is recessive, heterozygous carriers either show a reduced phenotype of partial lipodystrophy, pulmonary hypertension, or no phenotype. To investigate the pathogenic mechanisms underlying this syndrome in more depth, we performed next generation RNA sequencing of peripheral blood, which showed several dysregulated pathways in the patient that might be related to the phenotypic progeroid features (apoptosis, DNA repair/replication, mitochondrial. Secondly, we found a significant down-regulation of known Cav1 interaction partners, verifying the dysfunction of CAV1. Other known progeroid genes and lipodystrophy genes were also dysregulated. Next, western blotting of lysates of cultured fibroblasts showed that the patient shows a significantly decreased expression of wild-type CAV1 protein, demonstrating a loss-of-function mutation, though her phenotype is more severe that other heterozygotes with similar mutations. This phenotypic variety could be explained by differences in genetic background. Indications for this are supported by additional rare variants we found in AGPAT2 and LPIN1 lipodystrophy genes. CAV1, AGPAT2 and LPIN1 all play an important role in triacylglycerol (TAG biosynthesis in adipose tissue, and the defective function in different parts of this pathway, though not all to the same extend, could contribute to a more severe lipoatrophic phenotype in this patient. In conclusion, we report, for the first time, an association of CAV1 dysfunction

  12. Pathology of the parathyroid glands in hyperparathyroidism.

    Science.gov (United States)

    Baloch, Zubair W; LiVolsi, Virginia A

    2013-08-01

    This paper reviews the embryology, histology and pathology of the human parathyroid glands. It emphasizes those pathologic lesions which are found in the setting of clinical hyperparathyroidism. Also discussed are certain molecular features of hyperfunctioning parathyroid glands. The difficulties encountered in parathyroid FNA are reviewed and illustrated.

  13. Carcinoma of the parathyroid gland with hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Trevino Canamar, G.; Vogel, H.

    1983-02-01

    A patient with an endocrine-active carcinoma of the parathyroid gland was observed. The typical signs of hyperthyroidism could be seen in the skelettal system. Symptoms of bone and kidney diseases dominated the clinical picture. The symptomatology corresponded to a subchronic primary hyperparathyroidism.

  14. Oro-mandibular manifestations of primary hyperparathyroidism

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    Sachin Rai

    2012-01-01

    Conclusion : Loss of lamina dura, ground glass appearance, and mandibular cortical width reduction are common findings in primary hyperparathyroidism and these are significantly correlated with elevated parathormone and alkaline phosphatase. However, the presence of brown tumors and oral tori are less commonly encountered features.

  15. Consequences of long-term hyperparathyroidism

    NARCIS (Netherlands)

    Graal, M B; Wolffenbuttel, B H

    1998-01-01

    We describe a young woman with long-term untreated hyperparathyroidism with a superimposed vitamin D deficiency and an extremely decreased bone mineral density that was complicated by a vertebral fracture. Despite pretreatment with intravenous pamidronate and short-term vitamin D supplementation, se

  16. BMD improvements after operation for primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Rolighed, Lars; Vestergaard, Peter; Heickendorff, Lene

    2013-01-01

    PURPOSE: This study aims to quantify bone mineral density (BMD) changes following surgery in patients with primary hyperparathyroidism (PHPT) and to assess their relationship with clinical and biochemical variables. METHODS: A historic cohort of 236 PHPT patients with DXA scans pre- and 1-year...

  17. Cinacalcet for hyperparathyroidism in pregnancy and puerperium.

    NARCIS (Netherlands)

    Horjus, C.S.; Groot, I.; Telting, D.; Setten, P. van; Sorge, A. van; Kovacs, C.S.; Hermus, A.R.M.M.; Boer, H. de

    2009-01-01

    The efficacy and safety of various modes of medical treatment for primary hyperparathyroidism (PHPT) in pregnancy is largely unknown. This report describes two cases of PHPT in pregnancy that were temporarily treated with the calcimimetic cinacalcet. The first case was diagnosed in the 31st week of

  18. PHARMACODYNAMIC BEHAVIOR OF VECURONIUM IN PRIMARY HYPERPARATHYROIDISM

    NARCIS (Netherlands)

    ROLAND, EJL; WIERDA, JMKH; EURIN, BG; ROUPIE, E

    1994-01-01

    This study evaluated the potency and time course of action of vecuronium in patients with primary hyperparathyroidism (HPT) and marked hypercalcaemia during nitrous oxide-opioid anaesthesia. Twenty ASA physical status I and Il patients were studied by measuring the force of contraction of the adduct

  19. Incidence and Severity of Prescribing Errors in Parenteral Nutrition for Pediatric Inpatients at a Neonatal and Pediatric Intensive Care Unit

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    Theresa Hermanspann

    2017-06-01

    Full Text Available ObjectivesPediatric inpatients are particularly vulnerable to medication errors (MEs, especially in highly individualized preparations like parenteral nutrition (PN. Aside from prescribing via a computerized physician order entry system (CPOE, we evaluated the effect of cross-checking by a clinical pharmacist to prevent harm from PN order errors in a neonatal and pediatric intensive care unit (NICU/PICU.MethodsThe incidence of prescribing errors in PN in a tertiary level NICU/PICU was surveyed prospectively between March 2012 and July 2013 (n = 3,012 orders. A pharmacist cross-checked all PN orders prior to preparation. Errors were assigned to seven different error-type categories. Three independent experts from different academic tertiary level NICUs judged the severity of each error according to the National Coordinating Council for Medication Error Reporting and Prevention (NCC MERP Index (categories A–I.ResultsThe error rate was 3.9% for all 3,012 orders (118 prescribing errors in 111 orders. 77 (6.0%, 1,277 orders errors occurred in the category concentration range, all concerning a relative overdose of calcium gluconate for peripheral infusion. The majority of all events (60% were assigned to categories C and D (without major harmful consequences while 28% could not be assigned due to missing majority decision. Potential harmful consequences requiring interventions (category E could have occurred in 12% of assessments.ConclusionNext to systematic application of clinical guidelines and prescribing via CPOE, order review by a clinical pharmacist is still required to effectively reduce MEs and thus to prevent minor and major adverse drug events with the aim to enhance medication safety.

  20. Lithium-associated primary hyperparathyroidism complicated by nephrogenic diabetes insipidus.

    Science.gov (United States)

    Aksakal, Nihat; Erçetin, Candaş; Özçınar, Beyza; Aral, Ferihan; Erbil, Yeşim

    2015-01-01

    Lithium-associated hyperparathyroidism is the leading cause of hypercalcemia in lithium-treated patients. Lithium may lead to exacerbation of pre-existing primary hyperparathyroidism or cause an increased set-point of calcium for parathyroid hormone suppression, leading to parathyroid hyperplasia. Lithium may cause renal tubular concentration defects directly by the development of nephrogenic diabetes insipidus or indirectly by the effects of hypercalcemia. In this study, we present a female patient on long-term lithium treatment who was evaluated for hypercalcemia. Preoperative imaging studies indicated parathyroid adenoma and multinodular goiter. Parathyroidectomy and thyroidectomy were planned. During the postoperative course, prolonged intubation was necessary because of agitation and delirium. During this period, polyuria, severe dehydration, and hypernatremia developed, which responded to controlled hypotonic fluid infusions and was unresponsive to parenteral desmopressin. A diagnosis of nephrogenic diabetes insipidus was apparent. A parathyroid adenoma and multifocal papillary thyroid cancer were detected on histopathological examination. It was thought that nephrogenic diabetes insipidus was masked by hypercalcemia preoperatively. A patient on lithium treatment should be carefully followed up during or after surgery to prevent life-threatening complications of previously unrecognized nephrogenic diabetes insipidus, and the possibility of renal concentrating defects on long-term lithium use should be sought, particularly in patients with impaired consciousness.

  1. Severe fetal and neonatal hemolytic anemia due to a 198 kb deletion removing the complete β-globin gene cluster.

    Science.gov (United States)

    Verhovsek, Madeleine; Shah, Nirmish R; Wilcox, Ibifiri; Koenig, Sara C; Barros, Tiago; Thornburg, Courtney D; Steinberg, Martin H; Luo, Hong-yuan; Chui, David H K

    2012-11-01

    Fetal and neonatal hemolytic anemia can be caused by (γδβ)(0)-thalassemia deletions of the β-globin gene cluster. Many of these deletions have not been well characterized, and diagnostic tests are not readily available, thus hampering carrier detection, family counseling, and antenatal diagnosis. We report and define a 198 kb deletion removing the entire β-globin gene cluster, which was found in members of a multigeneration family of Irish/Scottish descent. The proband had life-threatening fetal and neonatal hemolytic anemia which subsided by 1 year of age. Copyright © 2012 Wiley Periodicals, Inc.

  2. Early-onset, severe lipoatrophy in a patient with permanent neonatal diabetes mellitus secondary to a recessive mutation in the INS gene.

    Science.gov (United States)

    Rachmiel, Marianna; Rubio-Cabezas, Oscar; Ellard, Sian; Hattersley, Andrew T; Perlman, Kusiel

    2012-09-01

    We describe a case of neonatal diabetes due to a homozygous mutation (c.3 G>T) at the INS gene, leading to lack of insulin expression and severe hyperglycemia from day one of life requiring permanent insulin replacement therapy. The genetic loss of endogenous insulin production likely led to lack of immune tolerance to insulin, with resultant autoantibody production against exogenous insulin and progressive immune-mediated lipoatrophy at injection sites.

  3. Neonatal swallowing assessment and practical recommendations for oral feeding in a girl with a severe congenital myopathy

    NARCIS (Netherlands)

    Engel-Hoek, L. van den; Erasmus, C.E.; Swart, B.J.M. de; Sie, L.T.L.; Groot, I.J.M. de

    2011-01-01

    The investigators report the case of a 6-week-old girl with a cap-like congenital myopathy and frequent coughing during feeding. Neonatal swallowing assessments were performed with surface electromyography of the submental muscle group and nasal airflow during sucking and swallowing. A

  4. Frequent large germline HRPT2 deletions in a French National cohort of patients with primary hyperparathyroidism.

    Science.gov (United States)

    Bricaire, Léopoldine; Odou, Marie-Françoise; Cardot-Bauters, Catherine; Delemer, Brigitte; North, Marie-Odile; Salenave, Sylvie; Vezzosi, Delphine; Kuhn, Jean-Marc; Murat, Arnaud; Caron, Philippe; Sadoul, Jean-Louis; Silve, Caroline; Chanson, Philippe; Barlier, Anne; Clauser, Eric; Porchet, Nicole; Groussin, Lionel

    2013-02-01

    Hyperparathyroidism-jaw tumor syndrome (HPT-JT) is an autosomal dominant syndrome with incomplete penetrance that can associate in a single patient parathyroid adenoma or carcinoma, fibro-osseous jaw tumor, cystic kidney lesion, and uterine tumor. Germline mutations of the HRPT2 gene (CDC73) coding for parafibromin are identified in approximately 50%-75% of HPT-JT cases and in approximately 14% of familial isolated hyperparathyroidism. A whole deletion of this gene has recently been reported in 1 sporadic case and in a family presenting with HPT-JT. The objective of the study was to report molecular abnormalities of the HRPT2 gene in patients with primary hyperparathyroidism in a French National cohort from the Groupe d'Étude des Tumeurs Endocrines. Patients' genomic DNA was screened by PCR-based sequencing for point mutations affecting HRPT2 and real-time quantitative PCR analysis for gross deletions. We report 20 index patients with a germinal HRPT2 abnormality. Median age at diagnosis of primary hyperparathyroidism was 23 years (range 14-65 years). Median serum total calcium level at diagnosis was 3.19 mmol/L (range 2.8-4.3 mmol/L). Thirteen different mutations were identified by routine sequencing, including 7 mutations never reported. Seven patients (35%) carried a gross deletion of this gene (3 complete and 4 partial deletions). No genotype-phenotype correlation could be identified. A gross deletion of the HRPT2 gene was identified in 7% of patients for whom a routine screening by direct sequencing came up as negative. Gross deletion analysis of the HRPT2 gene is indicated for all patients negative for mutation, presenting with HPT-JT or familial isolated hyperparathyroidism, parathyroid carcinoma, or in patients with apparently sporadic parathyroid adenoma diagnosed at a young age, having a severe hypercalcemia.

  5. Hyperparathyroidism with hypercalcaemia in chronic kidney disease: primary or tertiary?

    Science.gov (United States)

    Lunn, Mitchell R; Muñoz Mendoza, Jair; Pasche, Lezlee J; Norton, Jeffrey A; Ayco, Alexander L; Chertow, Glenn M

    2010-08-01

    Objective . This study aims to highlight the challenges in the diagnosis of hyperparathyroidism (HPT) in patients with advanced chronic kidney disease (CKD). Methods . In this report, we describe a middle-aged Filipino gentleman with underlying CKD who presented with intractable nausea, vomiting, severe and medically refractory hypercalcaemia and parathyroid hormone (PTH) concentrations in excess of 2400 pg/mL. The underlying pathophysiology as well as the aetiologies and current relevant literature are discussed. We also suggest an appropriate diagnostic approach to identify and promptly treat patients with CKD, HPT and hypercalcaemia. Results . Evaluation confirmed the presence of a large parathyroid adenoma; HPT and hypercalcaemia resolved rapidly following resection. Conclusion . This case report is remarkable for its severe hypercalcaemia requiring haemodialysis, large adenoma size, acute-on-chronic kidney injury and markedly elevated PTH concentration in association with primary HPT in CKD.

  6. Molecular Alterations in Sporadic Primary Hyperparathyroidism

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    Maria Inês Alvelos

    2011-01-01

    Full Text Available Primary hyperparathyroidism (PHPT is a frequent endocrine disorder characterized by an excessive autonomous production and release of parathyroid hormone (PTH by the parathyroid glands. This endocrinopathy may result from the development of a benign lesion (adenoma or hyperplasia or from a carcinoma. Most of the PHPT cases occur sporadically; however, approximately 10% of the patients present a familial form of the disease. The molecular mechanisms underlying the pathogenesis of sporadic PHPT are incompletely understood, even though somatic alterations in MEN1 gene and CCND1 protein overexpression are frequently observed. The MEN1 gene is mutated in about 30% of the parathyroid tumours and the protooncogene CCND1 is implicated in parathyroid neoplasia by rearrangements, leading to an overexpression of CCND1 protein in parathyroid cells. The aim of this work is to briefly update the molecular alterations underlying sporadic primary hyperparathyroidism.

  7. [Normocalcemic primary hyperparathyroidism: a growing problem].

    Science.gov (United States)

    Martínez Díaz-Guerra, Guillermo; Guadalix Iglesias, Sonsoles; Hawkins Carranza, Federico

    2013-08-04

    Normocalcemic primary hyperparathyroidism is at present one of the most common reasons for consultation in bone metabolism units. It is characterized by increased levels of intact parathyroid hormone in the presence of normal serum calcium (total and ionized) in generally asymptomatic individuals. The differential diagnosis should be considered in all situations that occur with secondary hyperparathyroidism. Its natural history is not well known, and it does not always progress to hypercalcemia. As a recently recognized entity, there are still no specific recommendations for its management. In this review we discuss some aspects of this entity, emphasizing the importance of a proper laboratory diagnosis, assessing possible signs or symptoms associated such as kidney stones or osteoporosis, which can help the clinician to take a conservative or interventionist attitude. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  8. Hyperparathyroidism in pregnancy: Report of two cases

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    Adem Güngör

    2013-09-01

    Full Text Available Primary hyperparathyroidism is a quite rare, life-threateningsituation for mother and fetus in pregnancy. The diagnosisis difficult when hyperemesis gravidarum is present.At diagnosis, the increase in PTH and calcium level andthe decrease in phosphorus level is quite important. Thegold Standard in radiological diagnosis is technetium-99msestamibi scintigraphy screening method. However, it iscontraindicated in pregnancy. Previously diagnosed pregnantwomen are given to surgery if they are symptomaticif they asymptomatic they are followed up until the end ofpregnancy by medical treatment. If it is possible surgicalprocedure most be performed in the second trimester ofpregnancy. In this report, two patients with hyperparathyroidismthat diagnosed during pregnancy are presented.Key words: Primary hyperparathyroidism, pregnancy,hyperemesis gravidarum

  9. Parathyroid and bone imaging in primary hyperparathyroidism

    Institute of Scientific and Technical Information of China (English)

    ZHU Rui-Sen; LU Han-Kui; LUO Quan-Yong; CHEN Li-Bo; MA Ji-Xiao

    2004-01-01

    Skeletal derangements occur quite often in patient with primary hyperparathyroidism (PHPT). We investigated parathyroid and bone imagings in 59 cases of pathologically proven PHPT. Forty-nine cases were pathologically proven parathyroid adenomas; 8 presented hyperplasia and the other 2 were adenocarcinomas. Parathyroid imaging (early phase imaging, EPI) was conducted at 30 min after injecting 740~925MBq 99mTc-MIBI and 2~3h later (delayed phase imaging, DPI) separately. The following thyroid imagings were performed at the same posture 10 min after intravenous injection of 74~111MBq 99mTcO4-. The 99mTc- MIBI subtraction imaging data were obtained by subtracting thyroid imaging from that of DPI. Among 49 cases of proven hyperparathyroid adenoma 45 yielded positive imagings. Eight cases with hyperplasia gave negative results. The results were positive in 2 cases of parathyroid adenocarcinoma. Results of 99mTc-MDP/bone imaging: 35 cases of hyperparathyroid adenocarcinoma (disease duration 1-6 months) showed normal bone images, while 14 cases showed superscan images, course being 4-12 months. Bone imaging for 2 cases of adenocarcinoma showed multiple, radioactive aggregated foci (brown tumor imaging); course lasting 10-24 months. The results of bone imaging in 8 cases of hyperplasia/ hyperparathyroidism were normal. It was concluded that diagnostic accuracy for parathyroid was 79.6% and for parathyroid adenoma was 91.8%, and the technique has no diagnostic value for hyperplasia. The 99mTc-MDP / bone imaging results for PHPT can be classified into three categories, i.e. normal, superscan and brown tumor. The imaging results correlated well with the different categories and degrees of bone damage, the duration of clinical course and the pathological types. Therefore, it's important to use bone imaging data in association with therapy to reflect the stage and progress of PHPT.

  10. Neonatal swallowing assessment and practical recommendations for oral feeding in a girl with a severe congenital myopathy.

    Science.gov (United States)

    van den Engel-Hoek, Lenie; Erasmus, Corrie E; de Swart, Bert J M; Sie, Lilian T L; de Groot, Imelda J M

    2011-08-01

    The investigators report the case of a 6-week-old girl with a cap-like congenital myopathy and frequent coughing during feeding. Neonatal swallowing assessments were performed with surface electromyography of the submental muscle group and nasal airflow during sucking and swallowing. A videofluoroscopic swallow study was performed separately. The registration of swallowing and breathing revealed a highly coordinated sequence of sucking, swallowing, and breathing. However, after a series of sucking and swallowing there was residue in the hypopharyngeal area, causing laryngeal penetration and coughing. Based on the findings, specific recommendations such as the use of a soft nipple, diminished suck-swallow sequences, and limited feeding time were made for this girl. These proved to be appropriate to prevent coughing as a signal of penetration or aspiration of milk during swallowing. This case highlights the profit of neonatal swallowing assessment, which can provide recommendations for safe oral feeding.

  11. Changes of several adrenal delta 4-steroids measured by HPLC-UV spectrometry in neonatal patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

    Science.gov (United States)

    Saisho, S; Shimozawa, K; Yata, J

    1990-01-01

    We have developed an easy and rapid method of reverse-phase high-performance liquid chromatography (HPLC)-UV spectrometry for measuring adrenal delta 4-steroids. Three female neonates with adrenal 21-hydroxylase deficiency (2 salt-losers and 1 simple virilizer), two of whom were recalled by neonatal mass-screening for congenital adrenal hyperplasia (CAH), were diagnosed using this method. Changes of several adrenal steroids were examined in these patients before and after treatment with hydrocortisone. Before treatment, the cortisone and cortisol peaks were very low and those of 17 alpha-hydroxyprogesterone (17-OHP) and 21-deoxycortisol (21-DOF) were high in all 3 patients (17-OHP: 79.9-997 nmol/l, 21-DOF: 83.7-324 nmol/l). The androstenedione peak was also high in 2 of them. A peak produced by 21-deoxycortisone, which is a product of oxidation of 21-DOF at the C-11 position, was also detected in all cases (14.5-297 nmol/l). After treatment, all of these abnormally elevated delta 4-steroids decreased or disappeared. This new method is thought to be valuable for the rapid diagnosis of CAH, and especially for use in neonatal mass-screening for CAH.

  12. The Development of Severe Neonatal Alloimmune Thrombocytopenia due to Anti-HPA-1a Antibodies Is Correlated to Maternal ABO Genotypes

    Directory of Open Access Journals (Sweden)

    Maria Therese Ahlen

    2012-01-01

    Full Text Available Background. Maternal alloantibodies against HPA-1a can cross placenta, opsonize foetal platelets, and induce neonatal alloimmune thrombocytopenia (NAIT. In a study of 100, 448 pregnant women in Norway during 1995–2004, 10.6% of HPA-1a negative women had detectable anti-HPA-1a antibodies. Design and Methods. A possible correlation between the maternal ABO blood group phenotype, or underlying genotype, and severe thrombocytopenia in the newborn was investigated. Results. We observed that immunized women with blood group O had a lower risk of having a child with severe NAIT than women with group A; 20% with blood group O gave birth to children with severe NAIT, compared to 47% among the blood group A mothers (relative risk 0.43; 95% CI 0.25–0.75. Conclusion. The risk of severe neonatal alloimmune thrombocytopenia due to anti-HPA-1a antibodies is correlated to maternal ABO types, and this study indicates that the observation is due to genetic properties on the maternal side.

  13. [PTHi value in the surgical treatment of hyperparathyroidism].

    Science.gov (United States)

    Ferrer Baixauli, F; Severá Ferrándiz, G; Menor Ibáñez, F; Infante Matarredona, E

    2004-01-01

    The prevalence of hyperparathyroidism as increased in the last 20 years due to a longer number of biochemical determinations, in which we observe in many cases hipercalcemia by casualty. The simptomatology of the primary hyperparathyroidism is very subtile and sometimes none in an 80% of the patients. Between november 1996 and march 2003 we have operated 55 parathyroidectomies. 36 patients (65,4%) had primary hyperparathyroidism, 16 (29,1%) secondary hyperparathyroidism and the rest (5,4%) tertiary hyperparathyroidism. Our hospital has a device of rapid detection of the parathyroid hormone level since february 2000, allowing us its satisfactory intraoperative determination in few minutes, evaluating the decrease to values inferior to the half of its basal level.

  14. Co-stimulation with LPS or Poly I:C markedly enhances the anti-platelet immune response and severity of fetal and neonatal alloimmune thrombocytopenia.

    Science.gov (United States)

    Li, Conglei; Chen, Pingguo; Vadasz, Brian; Ma, Li; Zhou, Hui; Lang, Sean; Freedman, John; Ni, Heyu

    2013-12-01

    Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a life-threatening bleeding disorder caused by maternal antibodies against fetal/neonatal platelets. FNAIT is also linked with miscarriages, although the incidence and mechanisms of fetal death have not been well studied. IntegrinαIIbβ3 (GPIIbIIIa) and the GPIbα complex are major glycoproteins expressed on platelets and are also major antigens targeted in autoimmune thrombocytopenia (ITP), but reported cases of anti-GPIb-mediated FNAIT are rare. Bacterial and viral infections have been causally linked with the pathogenesis of immune-mediated thrombocytopenia (ITP); however, it is unknown whether these infections contribute to the severity of FNAIT. Here, immune responses against platelet antigens were examined by transfusing wild-type (WT) mouse platelets into β3-/- or GPIbα-/- mice. To mimic bacterial or viral infections, lipopolysaccharide (LPS) or polyinosinic:polycytidylic acid (Poly I:C) were injected intraperitoneally following platelet transfusions. The FNAIT model was established by breeding the immunised female mice with WT male mice. We demonstrated for the first time that the platelet GPIbα has lower immunogenicity compared to β3 integrin. Interestingly, co-stimulation with LPS or Poly I:C markedly enhanced the immune response against platelet GPIbα and caused severe pathology of FNAIT (i.e. miscarriages). LPS or Poly I:C also enhanced the immune response against platelet β3 integrin. Our data suggest that bacterial and viral infections facilitate the anti-platelet GPIbα response, which may lead to a severe non-classical FNAIT (i.e. miscarriage but not neonatal bleeding) that has not been adequately reported in humans.

  15. Usefulness of ultrasonographic evaluation in primary and secondary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Jeon, Tae Joo; Kim, Eun Kyung; Lee, Jong Doo; Park, Jung Soo; Lee, Jong Tae; Yoo, Hyung Sik [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    1999-09-15

    To evaluate the accuracy and ultrasonographic findings of primary and secondary hyperparathyroidism (HPT) and correlate them with pathologic results. We reviewed 31 cases of surgically confirmed primary (n=22) and secondary (n=9) hyperparathyroidism. We used 10 or 7.5 MHz linear transducer and reviewed the location, contour, size and echogenicity of lesions. Then we evaluated the detection rate of parathyroid lesions based on surgical result and compared the result of {sup 99m}Tc-sestamibi scan (15 cases). Location of primary HPT was left lower in 9, left upper in 5, right lower in 4, right upper in 3, left midportion in 1 and superior mediastinum in 1. Lesions showed variable echogenicity-mild low echo (2), moderate low echo (10), severe low echo (2), isoecho (4) and heterogeneous echo pattern (1). All the lesions except 5 were well defined and 3 lesions had echogenic rim. Posterior enhancement and lateral shadowing were noted in 3 and 4 lesions, respectively. Nineteen of 23 primary lesions were detected by ultrasonography (82.6%) and well correlated with sestamibi scan. In case of secondary HPT, most were well defined low echoic nodular lesions, and we could detect 6 of 9 patients (67%) and 15 of 36 lesions (41.7%). Only 6 of 24 secondary lesion were detected by sestamibi scan (25%). The detection rate of ultrasonography in primary HPT was fairly good and well correlated with the result of the {sup 99m}Tc-sestamibi scan, but both diagnostic modalities were not promising in secondary HPT.

  16. Neonatal Venous Thromboembolism

    Directory of Open Access Journals (Sweden)

    Kristina M. Haley

    2017-06-01

    Full Text Available Neonates are the pediatric population at highest risk for development of venous thromboembolism (VTE, and the incidence of VTE in the neonatal population is increasing. This is especially true in the critically ill population. Several large studies indicate that the incidence of neonatal VTE is up almost threefold in the last two decades. Central lines, fluid fluctuations, sepsis, liver dysfunction, and inflammation contribute to the risk profile for VTE development in ill neonates. In addition, the neonatal hemostatic system is different from that of older children and adults. Platelet function, pro- and anticoagulant proteins concentrations, and fibrinolytic pathway protein concentrations are developmentally regulated and generate a hemostatic homeostasis that is unique to the neonatal time period. The clinical picture of a critically ill neonate combined with the physiologically distinct neonatal hemostatic system easily fulfills the criteria for Virchow’s triad with venous stasis, hypercoagulability, and endothelial injury and puts the neonatal patient at risk for VTE development. The presentation of a VTE in a neonate is similar to that of older children or adults and is dependent upon location of the VTE. Ultrasound is the most common diagnostic tool employed in identifying neonatal VTE, but relatively small vessels of the neonate as well as frequent low pulse pressure can make ultrasound less reliable. The diagnosis of a thrombophilic disorder in the neonatal population is unlikely to change management or outcome, and the role of thrombophilia testing in this population requires further study. Treatment of neonatal VTE is aimed at reducing VTE-associated morbidity and mortality. Recommendations for treating, though, cannot be extrapolated from guidelines for older children or adults. Neonates are at risk for bleeding complications, particularly younger neonates with more fragile intracranial vessels. Developmental alterations in the

  17. Preoperative diagnosis of primary hyperparathyroidism by MRI

    Energy Technology Data Exchange (ETDEWEB)

    Inouye, Takahiro; Tomita, Toshiki; Nitta, Seiichi [Tochigi Prefecture Saiseikai Utsunomiya Hospital (Japan); Kanou, Shigeru; Sato, Toshihiko

    1996-06-01

    We evaluated 12 patients with primary hyperparathyroidism by MRI. Nine patients presented parathyroid adenomas and the others hypertrophy of the parathyroid. Abnormal parathyroid was detected in 10 patients (83%) by T2-weighed image. And abnormal parathyroid was detected in one of the other two cases by MRI combined with {sup 99m}Tc{center_dot}MIBI-{sup 99m}Tc subtraction scintigraphy. Although we usually employ the axial view of MRI, it is incompatible with the operative field. We therefore hope that three-dimensional MRI will become compatible with the operative field in the future. (author)

  18. Primary Hyperparathyroidism: Effects on Bone Health.

    Science.gov (United States)

    Zanocco, Kyle A; Yeh, Michael W

    2017-03-01

    Primary hyperparathyroidism (PHPT) is the most common cause of chronic hypercalcemia. With the advent of routine calcium screening, the classic presentation of renal and osseous symptoms has been largely replaced with mild, asymptomatic disease. In hypercalcemia caused by PHPT, serum parathyroid hormone levels are either high, or inappropriately normal. A single-gland adenoma is responsible for 80% of PHPT cases. Less frequent causes include 4-gland hyperplasia and parathyroid carcinoma. Diminished bone mineral density and nephrolithiasis are the major current clinical sequelae. Parathyroidectomy is the only definitive treatment for PHPT, and in experienced hands, cure rates approach 98%.

  19. Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1.

    Science.gov (United States)

    Elaraj, Dina M; Skarulis, Monica C; Libutti, Steven K; Norton, Jeffrey A; Bartlett, David L; Pingpank, James F; Gibril, Fathia; Weinstein, Lee S; Jensen, Robert T; Marx, Stephen J; Alexander, H Richard

    2003-12-01

    Hyperparathyroidism in patients with multiple endocrine neoplasia type 1 (MEN1) is characterized by multiglandular disease and a propensity for recurrence after parathyroidectomy (PTx). This study analyzes outcomes of a cohort of MEN1 patients undergoing initial PTx at one institution. Between April 1960 and September 2002, 92 patients with MEN1 underwent initial PTx. Outcomes were analyzed based on extent of parathyroid resection. Fourteen percent had 2.5 or fewer glands resected, 69% had subtotal PTx, and 17% had total PTx (88% with immediate autotransplantation). The initial surgical cure rate was 98%. Excluding 6 patients lost to follow-up, 33% have developed recurrent hyperparathyroidism (in 46% after < or =2.5 PTx, in 33% after subtotal, and in 23% after total PTx). Median recurrence-free survival was not statistically significantly different between subtotal versus total PTx, but it was longer for subtotal and total PTx compared with lesser resection (16.5 vs 7.0 years, respectively, P=.03). The incidence of severe hypoparathyroidism was 46% after total versus 26% after subtotal PTx. Subtotal and total PTx result in durable control of MEN1-associated hyperparathyroidism and have longer recurrence-free intervals compared with lesser resection. The high incidence of severe hypoparathyroidism after total PTx suggests that subtotal PTx is the initial operation of choice in this setting.

  20. Serious adverse neonatal outcomes such as 5-minute Apgar score of zero and seizures or severe neurologic dysfunction are increased in planned home births after cesarean delivery.

    Science.gov (United States)

    Grünebaum, Amos; McCullough, Laurence B; Arabin, Birgit; Chervenak, Frank A

    2017-01-01

    The United States is with 37,451 home births in 2014 the country with the largest absolute number of home births among all developed countries. The purpose of this study was to examine the occurrence and risks of a 5-minute Apgar score of zero and neonatal seizures or serious neurologic dysfunction in women with a history of prior cesarean delivery for planned home vaginal birth after cesarean (VBAC), compared to hospital VBAC and hospital birth cesarean deliveries for term normal weight infants in the United States from 2007-2014. We report in this study outcomes of women who had one or more prior cesarean deliveries and included women who had a successful vaginal birth after a trial of labor after cesarean (TOLAC) at home and in the hospital, and a repeat cesarean delivery in the hospital. We excluded preterm births (Apgar score of 0 of 1 in 890 (11.24/10,000, relative risk 9.04, 95% confidence interval 4-20.39, p<.0001) and an incidence of neonatal seizures or severe neurologic dysfunction of 1 in 814 (Incidence: 12.27/10,000, relative risk 11.19, 95% confidence interval 5.13-24.29, p<.0001). Because of the significantly increased neonatal risks, obstetric providers should therefore not offer or perform planned home TOLACs and for those desiring a VBAC should strongly recommend a planned TOLAC in the appropriate hospital setting. We emphasize that this stance should be accompanied by effective efforts to make TOLAC available in the appropriate hospital setting.

  1. Severely impaired health status at diagnosis of Pompe disease: a cross-sectional analysis to explore the potential utility of neonatal screening.

    Science.gov (United States)

    Rigter, Tessel; Weinreich, Stephanie S; van El, Carla G; de Vries, Juna M; van Gelder, Carin M; Güngör, Deniz; Reuser, Arnold J J; Hagemans, Marloes L C; Cornel, Martina C; van der Ploeg, Ans T

    2012-11-01

    Since the introduction of enzyme replacement therapy for Pompe disease, awareness and early diagnosis have gained importance. Because the therapy is most effective when started early and methods for dried bloodspot screening for Pompe disease are currently being explored, neonatal screening is getting increased attention. The objective of this study was to investigate the gains that might be achieved with earlier diagnosis by neonatal screening. For this purpose we analyzed the health and functional status of non-screened patients with Pompe disease at the time of diagnosis. Previously collected clinical data and results of an international patient-reported questionnaire were used. Cross-sectional data of 53 patients with Pompe disease diagnosed between 1999 and 2009 (aged 0-64 years) were analyzed. According to the World Health Organization's International Classification of Functioning, Disability and Health the following domains are described: body function, activity, participation and contextual factors. In all patients with classic infantile Pompe disease cardiac function, hearing, muscle strength and motor development were considerably impaired at the time of clinical diagnosis. The use of oxygen and/or nasogastric tube-feeding was reported in more than 70% of these cases. Most children, adolescents and adults had advanced muscle weakness and impaired respiratory function at the time of their diagnosis, causing varying degrees of handicap. About 12% of them used a walking device and/or respiratory support at the time of diagnosis. The severely impaired health status reported here provides a strong argument for earlier diagnosis and to further explore the potential of neonatal screening for Pompe disease.

  2. Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center

    Directory of Open Access Journals (Sweden)

    Fabio Luiz de Menezes Montenegro

    2012-01-01

    Full Text Available Most cases of sporadic primary hyperparathyroidism present disturbances in a single parathyroid gland and the surgery of choice is adenomectomy. Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1 is an asynchronic, asymmetrical multiglandular disease and it is surgically approached by either subtotal parathyroidectomy or total parathyroidectomy followed by parathyroid auto-implant to the forearm. In skilful hands, the efficacy of both approaches is similar and both should be complemented by prophylactic thymectomy. In a single academic center, 83 cases of hyperparathyroidism/ multiple endocrine neoplasia type 1 were operated on from 1987 to 2010 and our first surgical choice was total parathyroidectomy followed by parathyroid auto-implant to the non-dominant forearm and, since 1997, associated transcervical thymectomy to prevent thymic carcinoid. Overall, 40% of patients were given calcium replacement (mean intake 1.6 g/day during the first months after surgery, and this fell to 28% in patients with longer follow-up. These findings indicate that several months may be needed in order to achieve a proper secretion by the parathyroid auto-implant. Hyperparathyroidism recurrence was observed in up to 15% of cases several years after the initial surgery. Thus, long-term follow-up is recommended for such cases. We conclude that, despite a tendency to subtotal parathyroidectomy worldwide, total parathyroidectomy followed by parathyroid auto-implant is a valid surgical option to treat hyperparathyroidism/multiple endocrine neoplasia type 1. Larger comparative systematic studies are needed to define the best surgical approach to hyperparathyroidism/multiple endocrine neoplasia type 1.

  3. Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center

    Science.gov (United States)

    de Menezes Montenegro, Fabio Luiz; Lourenço, Delmar Muniz; Tavares, Marcos Roberto; Arap, Sergio Samir; Nascimento, Climerio Pereira; Neto, Ledo Mazzei Massoni; D'Alessandro, André; Toledo, Rodrigo Almeida; Coutinho, Flávia Lima; Brandão, Lenine Garcia; de Britto e Silva Filho, Gilberto; Cordeiro, Anói Castro; Toledo, Sergio Pereira Almeida

    2012-01-01

    Most cases of sporadic primary hyperparathyroidism present disturbances in a single parathyroid gland and the surgery of choice is adenomectomy. Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical multiglandular disease and it is surgically approached by either subtotal parathyroidectomy or total parathyroidectomy followed by parathyroid auto-implant to the forearm. In skilful hands, the efficacy of both approaches is similar and both should be complemented by prophylactic thymectomy. In a single academic center, 83 cases of hyperparathyroidism/multiple endocrine neoplasia type 1 were operated on from 1987 to 2010 and our first surgical choice was total parathyroidectomy followed by parathyroid auto-implant to the non-dominant forearm and, since 1997, associated transcervical thymectomy to prevent thymic carcinoid. Overall, 40% of patients were given calcium replacement (mean intake 1.6 g/day) during the first months after surgery, and this fell to 28% in patients with longer follow-up. These findings indicate that several months may be needed in order to achieve a proper secretion by the parathyroid auto-implant. Hyperparathyroidism recurrence was observed in up to 15% of cases several years after the initial surgery. Thus, long-term follow-up is recommended for such cases. We conclude that, despite a tendency to subtotal parathyroidectomy worldwide, total parathyroidectomy followed by parathyroid auto-implant is a valid surgical option to treat hyperparathyroidism/multiple endocrine neoplasia type 1. Larger comparative systematic studies are needed to define the best surgical approach to hyperparathyroidism/multiple endocrine neoplasia type 1. PMID:22584718

  4. Review of cinacalcet hydrochloride in the management of secondary hyperparathyroidism.

    Science.gov (United States)

    Yousaf, Farhanah; Charytan, Chaim

    2014-02-01

    Cinacalcet is the first Food and Drug Administration-approved calcimimetic for the treatment of secondary hyperparathyroidism in dialysis patients. It is effective in improving control of parathyroid hormone, serum calcium, phosphorus, and calcium-phosphorus product. The calcium-lowering effect of cinacalcet overcomes the limitations of standard therapy associated hypercalcemia. There is evidence to suggest that cinacalcet has important clinical implications, which extend beyond its relevance in the treatment of secondary hyperparathyroidism. This review summarizes the evidence regarding the role of cinacalcet in the treatment of secondary hyperparathyroidism, disrupted bone mineral metabolism, cardiovascular disease, and mortality. In addition, the cost implications of cinacalcet are briefly explored.

  5. Ethical and legal dilemmas around termination of pregnancy for severe fetal anomalies: A review of two African neonates presenting with ventriculomegaly and holoprosencephaly.

    Science.gov (United States)

    Chima, S C; Mamdoo, F

    2015-12-01

    Termination of pregnancy (TOP) or feticide for severe fetal anomalies is ethically and morally challenging and maybe considered illegal in countries with restrictive abortion laws. While diagnostic modalities such as fetal ultrasound, magnetic resonance imaging, and genetic screening have improved prenatal diagnosis, these technologies remain scarce in many African countries making diagnosis and counseling regarding TOP difficult. Ethical dilemmas such as women's autonomy rights may conflict with fetus' right to personhood, and doctor's moral obligations to society. In liberal jurisdictions, previable fetuses may not have legal rights of personhood; therefore, appropriate action would be to respect pregnant women's decisions regarding TOP. However, in countries with restrictive abortion laws the fetus maybe imbued with the right of personhood at conception, making TOP illegal and exposing doctors and patients to potential criminal prosecution. Birth of a severely disabled baby with independent legal rights creates further conflicts between parents and clinicians complicating healthcare decision-making. Irrespective of the maternal decision to accept or refuse TOP, the psychological and emotional impact of an impaired fetus or neonate, often lead to moral distress and posttraumatic stress reactions in parents. Doctors have legal and ethical obligations to provide an accurate antenatal diagnosis with full disclosure to enable informed decision making. Failure to provide timely or accurate diagnosis may lead to allegations of negligence with potential liability for "wrongful birth" or "wrongful life" following birth of severely disabled babies. Mismanagement of such cases also causes misuse of scarce healthcare resources in resource-poor countries. This paper describes ethical challenges in clinical management of two neonates born following declined and failed feticide for severe central nervous system anomalies with a critical appraisal of the relevant literature.

  6. Neurobehavioral conditions and effects of gender, weight and severity in preterm infants according to the Neonatal Behavioral Assessment Scale

    Directory of Open Access Journals (Sweden)

    Alicia Álvarez-García

    2015-10-01

    Full Text Available The increasing number of preterm babies in recent years has raised interest in studying the consequences of prematurity as a risk factor. In the present paper, 30 preterm babies (at 40 weeks of gestational age were assessed using the Neonatal Behavioral Assessment Scale and the results were compared with those of a control group of 28 full term babies. Moreover, the influence of weight, sex and gestational age was analyzed considering the Brazelton results in the preterm group. The preterm group showed significantly lower scores than the control group for 9 of the 28 behavioral items in the Scale and for 2 of the 5 clusters. However, preterm babies performed better in habituation to disturbing stimuli (light and noise during sleep. In relation to the influence of sex, premature girls performed better in the Social-Interactive cluster. The preterm group has lower neurobehavioral conditions than the full term group, probably due to the abrupt interruption of their intrauterine maturation. In contrast, they showed a better ability of habituation, maybe as a consequence of a learning effect due to earlier additional extrauterine exposition.

  7. Hypocalcemic tetany in the newborn as a manifestation of unrecognized maternal primary hyperparathyroidism.

    Science.gov (United States)

    Pieringer, Herwig; Hatzl-Griesenhofer, Margit; Shebl, Omar; Wiesinger-Eidenberger, Gabriele; Maschek, Wilhelmine; Biesenbach, Georg

    2007-01-01

    Primary hyperparathyroidism (PHP) during pregnancy is a very rare event that increases maternal and perinatal morbidity and mortality. We present a case in which hypocalcemic tetany of the neonatal infant - caused by transient hypoparathyroidism in the child - finally revealed asymptomatic maternal PHP. An apparently healthy 30-year-old woman had an uneventful pregnancy and delivery. On the 15th postpartal day, the newborn developed hypocalcemic tetany. After receiving supplementation of calcium and vitamin D, the child developed without further pathological findings. Laboratory and radiological studies in the mother led to a diagnosis of maternal PHP. An adenoma of the right lower parathyroid gland was subsequently removed. The search for the cause of hypocalcemia in a newborn should not focus on the patient alone. Examining the apparently healthy mother and approaching the case in a multidisciplinary fashion may benefit both the child and the mother.

  8. 18F-FET-PET in Primary Hyperparathyroidism

    DEFF Research Database (Denmark)

    Krakauer, Martin; Kjær, Andreas; Bennedbæk, Finn Noe

    2016-01-01

    Preoperative localisation of the diseased parathyroid gland(s) in primary hyperparathyroidism (PHP) is a prerequisite for subsequent minimally invasive surgery. Recently, as alternatives to conventional sestamibi parathyroid scintigraphy, the (11)C-based positron emission tomography (PET) tracers...

  9. Further insights into the pathogenesis of primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Rejnmark, Lars; Amstrup, Anne Kristine; Mollerup, Charlotte

    2013-01-01

    CONTEXT: The pathogenesis of primary hyperparathyroidism (PHPT) is largely unknown. OBJECTIVE: The objective of the study was to ascertain the plasma levels of calcium, PTH, and 25-hydroxyvitamin D (25OHD) as measured prior to a clinical diagnosis of PHPT. STUDY SUBJECTS: Within three population......, undiagnosed PHPT was present in 63% of the cases. Among those without PHPT at inclusion (n = 43), 55% had normocalcemic hyperparathyroidism (vs. 21% in the matched controls, P ... controls, 25OHD levels were lower in normocalcemic hyperparathyroidism but not in normoparathyroid hypercalcemia. An adenoma was removed from 78% of the cases with normocalcemic hyperparathyroidism, whereas 39% of the cases with normoparathyroid hypercalcemia had parathyroid hyperplasia (P = 0.02). Overlap...

  10. Risk factors for treatment failure in surgery for primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Madsen, Anders Rørbæk; Rasmussen, Lars; Godballe, Christian

    2016-01-01

    Surgery for primary hyperparathyroidism (pHPT) has a high cure-rate and few complications. Preoperative localization procedures have permitted a dramatic shift from routine bilateral exploration to focused, minimally invasive procedures. At Odense University Hospital, Denmark, the introduction...

  11. Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1

    Directory of Open Access Journals (Sweden)

    Grzegorz Piecha

    2010-01-01

    Full Text Available Primary hyperparathyroidism may occur as a part of an inherited syndrome in a combination with pancreatic endocrine tumours and/or pituitary adenoma, which is classified as Multiple Endocrine Neoplasia type 1 (MEN-1. This syndrome is caused by a germline mutation in MEN-1 gene encoding a tumour-suppressor protein, menin. Primary hyperparathyroidism is the most frequent clinical presentation of MEN-1, which usually appears in the second decade of life as an asymptomatic hypercalcemia and progresses through the next decades. The most frequent clinical presentation of MEN-1-associated primary hyperparathyroidism is bone demineralisation and recurrent kidney stones rarely followed by chronic kidney disease. The aim of this paper is to present the pathomechanism, screening procedures, diagnosis, and management of primary hyperparathyroidism in the MEN-1 syndrome. It also summarises the recent advances in the pharmacological therapy with a new group of drugs—calcimimetics.

  12. Primary hyperparathyroidism having multiple Brown tumors mimicking malignancy

    Directory of Open Access Journals (Sweden)

    Ekram Ullah

    2012-01-01

    Full Text Available Primary hyperparathyroidism is a disease characterized by excessive secretion of parathormone. During the course of this disease, bone loss occurs, particularly depending on resorption of the skeletal system. One of the complications of primary hyperparathyroidism is fibrotic, cystic bony changes which is called Brown tumor. Skeletal manifestations in the form of Brown tumors are rare and according to literature occur in less than 2% of patients suffering from any form of hyperparathyroidism. Such rare and multiple benign lesions may simulate a malignant neoplasm and pose a real challenge for the clinician in its differential diagnosis. We present a case of a 23-year-old Indian woman who was evaluated for multiple lytic expansile lesions with a strong suspicion of malignancy and fibrous dysplasia but turned out to be a case of primary hyperparathyroidism.

  13. Kidney stones and crushed bones secondary to hyperparathyroidism.

    Science.gov (United States)

    Sreelesh, K P; Sreejith, G Nair; Pranab, K Prabhakaran

    2016-01-01

    Here we report a 65-year-old woman with multiple brown tumors and renal stones secondary to primary hyperparathyroidism. This case highlights the need for early recognition of parathyroid hyperactivity.

  14. [Normocalcemic primary hyperparathyroidism: recommendations for management and follow-up].

    Science.gov (United States)

    Martínez Díaz-Guerra, Guillermo; Jódar Gimeno, Esteban; Reyes García, Rebeca; Gómez Sáez, José Manuel; Muñoz-Torres, Manuel

    2013-10-01

    To provide practical recommendations for evaluation and follow-up of patients with normocalcemic primary hyperparathyroidism. Members of the Bone Metabolism Working Group of the Spanish Society of Endocrinology. A systematic search was made in MEDLINE (PubMed), using the terms normocalcemic primary hyperparathyroidism and primary hyperparathyroidism, for articles in English published before 22 November 2012. Literature was reviewed by 2 members of the Bone Metabolism Working Group of the Spanish Society of Endocrinology, and after development of recommendations, the manuscript was reviewed by all other members of the Group, and their suggestions were incorporated. The document provides practical recommendations for evaluation and follow-up of patients with normocalcemic primary hyperparathyroidism. There is however little evidence available about different aspects of this disease, mainly progression rate and clinical impact. More data are therefore needed before definite recommendations may be made. Copyright © 2012 SEEN. Published by Elsevier Espana. All rights reserved.

  15. Parathyroid Apoptosis and Proliferation in Uremic Patients with Severe Hyperparathyroidism

    Institute of Scientific and Technical Information of China (English)

    周富华; 王笑云

    1999-01-01

    IntroductionItiswelknownthatsecondaryhyper-parathyroidism(SHPT)isinducedbychronicrenalfailure(CRF),butthepathogenesisandprogr...

  16. Persistent renal hyperparathyroidism caused by intrathyroidal parathyroid glands.

    Science.gov (United States)

    Chen, Chin-Li; Lin, Shih-Hua; Yu, Jyh-Cherng; Shih, Ming-Lang

    2014-09-01

    Renal hyperparathyroidism usually occurs in chronic renal failure patients on regular dialysis. However, renal hyperparathyroidism resulting from intrathyroidal parathyroid glands is an uncommon condition. We herein present the case of a 35-year-old woman who has been on hemodialysis for 20 years. She had renal hyperparathyroidism with generalized weakness and bone pain for 2 years. The patient initially underwent parathyroidectomy at a local institution, during which two large parathyroid glands were resected from the right side (no parathyroid glands were found on the left side); however, the surgical procedure was unsuccessful, and the patient had persistent renal hyperparathyroidism after the operation. She was then transferred to our hospital and ectopic intrathyroidal parathyroid glands were localized by neck ultrasonography and technetium-99m sestamibi scans with single-photon emission computed tomography imaging preoperatively. A left thyroid lobectomy was performed and two intrathyroidal parathyroid glands were found. The patient recovered uneventfully and her symptoms resolved. Therefore, clinicians should be aware of the possibility of renal hyperparathyroidism resulting from intrathyroidal parathyroid glands in cases where the renal hyperparathyroidism persists after parathyroidectomy.

  17. Palpation thyroiditis following subtotal parathyroidectomy for hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Elizabeth M Madill

    2016-07-01

    Full Text Available Thyrotoxicosis is an under-recognised but clinically important complication of parathyroidectomy. We report a case of a 37-year-old man with tertiary hyperparathyroidism who initially developed unexplained anxiety, diaphoresis, tachycardia, tremor and hyperreflexia one day after subtotal parathyroidectomy. Thyroid biochemistry revealed suppressed thyroid stimulating hormone and elevated serum free T4 and free T3 levels. Technetium-99m scintigraphy scan confirmed diffusely decreased radiotracer uptake consistent with thyroiditis. The patient was diagnosed with thyrotoxicosis resulting from palpation thyroiditis. Administration of oral beta-adrenergic antagonists alleviated his symptoms and there was biochemical evidence of resolution fourteen days later. This case illustrates the need to counsel patients about thyroiditis as one of the potential risks of parathyroid surgery. It also emphasises the need for biochemical surveillance in patients with unexplained symptoms in the post-operative period and may help to minimise further invasive investigations for diagnostic clarification.

  18. Primary hyperparathyroidism: intraoperative PTH-measurements

    DEFF Research Database (Denmark)

    Rolighed, L; Heickendorff, L; Hessov, I

    2004-01-01

    measurement as a predictor of successful cure. MATERIAL AND METHODS: From September 1999 to April 2002 143 patients with pHPT underwent a parathyroid operation (bilateral neck exploration with identification of all parathyroid glands) with intraoperative measurements of plasma PTH (immediately prior......BACKGROUND: With the development of rapid assays and intraoperative measurement of intact parathyroid hormone (PTH), new strategies in the handling of patients with primary hyperparathyroidism (pHPT) have evolved. AIM: The aim of our study was to illustrate the performance of the intraoperative PTH...... to surgery (T0) and 5 minutes after gland excision (T5)). A positive test result was defined as plasma PTH values at T5 below 20% of T0 or a value in the normal range below 7.6 pmol/l. Hence T5 values above 20% of T0 and above 7.6 pmol/l were considered test negative. RESULTS: 122 patients (85%) were test...

  19. Hyperparathyroidism revisited - Old wine in new bottles!

    Directory of Open Access Journals (Sweden)

    Rekha Arcot

    2011-01-01

    Full Text Available Aim: Hyperparathyroidism (HPT is a condition that occurs due to exacerbated activity of the parathyroid glands. According to the etiology it may be primary, secondary or tertiary hyperparathyroidism (pHPT, sHPT, tHPT. This is a study done to document and evaluate the presentations of primary and secondary HPT, with the associated complications and the approach to management in these patients, at our hospital. Materials and Methods: Twenty-one patients with HPT were encountered at Sri Ramachandra Medical College and Research Institute between January 2000 and January 2010. Operative notes, histopathology files, and medical records were used for the retrospective analysis of the patients with HPT. Parathormone, calcium, and phosphate levels were estimated on all the patients, to determine the primary or secondary etiology of this endocrine abnormality. Furthermore, these patients were subjected to ultrasonography (USG of the neck and Technetium (99 mTc scan of the neck to identify the parathyroid gland. Results: This study revealed that about 76, 19, and 5% of the patients suffered from pHPT, sHPT, and tHPT, respectively, with a female preponderance (62%.The neoplasm in all patients with pHPT was parathyroid adenoma. The patients presented with renal, bony, and menstrual abnormalities. Cases with sHPT had a 15 - 20 year history of chronic kidney disease and they subsequently developed bony abnormalities. Even as all the patients with pHPT were managed with parathyroidectomy, individuals with sHPT were treated conservatively. Postoperative features of hypocalcemia were noted in only one patient. Conclusion: This study re-emphasizes that pHPT is more common and is often due to an adenoma. Recent advances in parathormone sampling operatively and minimal access surgery, along with accurate and prompt clinical diagnosis, is necessary for the cure of these patients presenting with obscure abdominal, bony, and renal ailments.

  20. A severe case of astrogliosis and encephalomalacia in a neonate with a massive vein of Galen malformation (VGM

    Directory of Open Access Journals (Sweden)

    F Ismail

    2011-06-01

    Full Text Available Vein of Galen malformations (VGMs are rare anomalies of intracranial circulation that constitute 1% of all intracranial vascular malformations. We describe a case of severe encephalomalacia associated with a VGM, which has not been previously described.

  1. New options for the management of hyperparathyroidism after renal transplantation.

    Science.gov (United States)

    Douthat, Walter Guillermo; Chiurchiu, Carlos Raul; Massari, Pablo Ulises

    2012-06-24

    The persistence and severity of hyperparathyroidism (HPT) post-renal transplantation is relatively frequent and primarily associated with the timing and its magnitude in the pre-transplant period and with the presence of parathyroid adenomas. HPT after renal transplantation is clinically manifested with hypercalcemia, hypophosphatemia, bone pain, fractures, and in more serious cases with cardiovascular calcifications that affect the survival. The primary clinical objective for patients with secondary HPT after renal transplantation is to obtain a level of parathyroid hormone (PTH) adequate to the renal transplanted function and to normalize levels of calcium, phosphorus and vitamin D. In many cases during this period, the development of hypercalcemia and/or hypophosphatemia makes it necessary to take different therapeutic measures. The use of vitamin D or its analogues has been extrapolated from the management of pre-transplant HPT obtaining variable outcomes, although its use is limited by its capacity to produce hypercalcemia. Calcimimetics are drugs that have proven be effective in reducing PTH levels in patients with HPT on dialysis and has been effective in reducing up to 50% PTH levels in moderate to severe HPT in post-renal transplantation.When HPT persists after renal transplantation and does not respond to medical treatment, invasive management by percutaneous ethanol injection therapy of parathyroid glands or parathyroidectomy should be considered. The emergence of new methods for the management of HPT expands the availability of therapeutic tools for transplant patients.

  2. Fibrous dysplasia of the jaws associated with secondary hyperparathyroidism: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Whi, Jung Hyun; Kim, Young Joo; Chun, Kyung Ah; Kim, Ki Tae; Chang, Eun Deok; Kim, Young Ok; Lee, Won [The Catholic University of Korea, Uijongbu (Korea, Republic of)

    2007-06-15

    There have been few reports on fibrous dyplasia associated with secondary hyperparathyroidism. We report a case of a hemodialysis patient with secondary hyperparathyroidism concomitant with fibrous dysplasia of the jaws causing an abnormal deformity.

  3. Interrelationship Between Broadband NIRS Measurements of Cerebral Cytochrome C Oxidase and Systemic Changes Indicates Injury Severity in Neonatal Encephalopathy.

    Science.gov (United States)

    Bale, Gemma; Mitra, Subhabrata; de Roever, Isabel; Chan, Marcus; Caicedo-Dorado, Alexander; Meek, Judith; Robertson, Nicola; Tachtsidis, Ilias

    2016-01-01

    Perinatal hypoxic ischaemic encephalopathy (HIE) is associated with severe neurodevelopmental problems and mortality. There is a clinical need for techniques to provide cotside assessment of the injury extent. This study aims to use non-invasive cerebral broadband near-infrared spectroscopy (NIRS) in combination with systemic physiology to assess the severity of HIE injury. Broadband NIRS is used to measure the changes in haemodynamics, oxygenation and the oxidation state of cytochrome c oxidase (oxCCO). We used canonical correlation analysis (CCA), a multivariate statistical technique, to measure the relationship between cerebral broadband NIRS measurements and systemic physiology. A strong relationship between the metabolic marker, oxCCO, and systemic changes indicated severe brain injury; if more than 60 % of the oxCCO signal could be explained by the systemic variations, then the neurodevelopmental outcome was poor. This boundary has high sensitivity and specificity (100 and 83 %, respectively). Broadband NIRS measured concentration changes of the oxidation state of cytochrome c oxidase has the potential to become a useful cotside tool for assessment of injury severity following hypoxic ischaemic brain injury.

  4. Utility of an intraoperabive gamma probe in the surgical management of secondary or tertiary hyperparathyroidism

    NARCIS (Netherlands)

    Jorna, Francisca H.; Jager, Pieter L.; Lemstra, Clara; Wiggers, Theo; Stegeman, Coen A.; Plukker, John T. M.

    2008-01-01

    BACKGROUND: In primary hyperparathyroidism the gamma probe is effective, but its role in secondary hyperparathyroidism is unclear. We investigated the utility of the probe in the surgical management of secondary and tertiary hyperparathyroidism. METHODS: The value of the probe in guiding resection o

  5. A severe neonatal lymphopenia associated with administration of azathioprine to the mother in a context of Crohn's disease.

    Science.gov (United States)

    Thomas, C; Monteil-Ganiere, C; Mirallié, S; Hémont, C; Dert, C; Léger, A; Joyau, C; Caldari, D; Audrain, M

    2017-09-02

    Azathioprine is commonly used in Crohn's Diseases. It has been administered to many pregnant women over many years without significant side effects. However, pancytopenia and Severe Combined Immune Deficiency-like disease have been reported in infants whose mothers received azathioprine throughout pregnancy. Moreover, myelotoxicity has been described in patients treated with azathioprine and having a low or absent thiopurine S-methyl transferase (TPMT) activity.Here, we describe the case of a new-born girl found highly lymphopenic (cells) after a positive new-born screening for Severe Combined Immuno Deficiency. The clinical examination was normal. The mother was being treated with azathioprine throughout her pregnancy without any reduction of the dose. It proved to be that the mother was heterozygous for the 3A (TPMT) activity mutation and that the baby was homozygous for the same mutation. 6-thioguanine nucleotides were high (744 pmol/8.10 8 RBC) in the mother and detectable in the infant (177 pmol/8.10 8 RBC).Although rare, this case illustrates the potential grave consequences of unsuspected TPMT homozygosity in a newborn of a mother receiving thiopurines during pregnancy. Because of the severity of the risk for the newborn, consideration should be given to performing mother genetic testing and newborn routine blood count in cases of thiopurines treatment during pregnancy. Copyright © 2017 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.

  6. Intraoperative nuclear guidance in benign hyperparathyroidism and parathyroid cancer

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    Bonjer, H.J. [Univ. Hospital Dijkzigt, Rotterdam (Netherlands). Dept. of Surgery; Bruining, H.A. [Univ. Hospital Dijkzigt, Rotterdam (Netherlands). Dept. of Surgery; Pols, H.A.P. [Univ. Hospital Dijkzigt, Rotterdam (Netherlands). Dept. of Internal Medicine 3; Herder, W.W. de [Univ. Hospital Dijkzigt, Rotterdam (Netherlands). Dept. of Internal Medicine 3; Eijck, C.H.J. [Univ. Hospital Dijkzigt, Rotterdam (Netherlands). Dept. of Surgery; Breeman, W.A.P. [Univ. Hospital Dijkzigt, Rotterdam (Netherlands). Dept. of Nuclear Medicine; Krenning, E.P. [Univ. Hospital Dijkzigt, Rotterdam (Netherlands). Dept. of Nuclear Medicine

    1997-03-01

    The success of parathyroid surgery is determined by the identification and removal of all hyperactive parathyroid tissue. Ectopic location of parathyroid tumours and fibrosis due to previous operations can cause failure of parathyroidectomy. Parathyroid tumours accumulate and retain 2-methoxyisobutylisonitrile (MIBI) labelled with technetium-99m. This study assesses the value of intra-operative localization of parathyroid tumours using a hand-held gamma detector in patients with hyperparathyroidism and parathyroid cancer. Twenty patients undergoing their first operations for hyperparathyroidism, 15 patients undergoing reoperations for either persistent or recurrent hyperparathyroidism and two patients with parathyroid cancer were studied. Radioactivity in the neck and the mediastinum was recorded by a gamma detector after administration of 370 MBq {sup 99m}Tc-MIBI. Surgical findings and postoperative serum levels of calcium were documented. The sensitivity of the gamma detector in identifying parathyroid tumours was 90.5% in first parathyroidectomies, 88.9% in reoperations for either persistent or recurrent hyperparathyroidism and 100% in parathyroid cancer. One false-positive result was due to a thyroid nodule. Hypercalcaemia ceased in all but one patient postoperatively. It is concluded that employment of the gamma detector is to be advocated in first parathyroidectomies when a parathyroid tumour cannot be discovered, in reoperations for either persistent or recurrent hyperparathyroidism and in surgery for parathyroid cancer. (orig.)

  7. Clinical Use of Cinacalcet in MEN1 Hyperparathyroidism

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    V. J. Moyes

    2010-01-01

    Full Text Available Background. Management of multiple-endocrine neoplasia type 1- (MEN1- associated hyperparathyroidism is associated with high recurrence rates and high surgical morbidity due to multiple neck explorations. Cinacalcet, a calcimimetic agent licensed for the treatment of secondary hyperparathyroidism and parathyroid carcinoma, may provide a medical alternative for the management of these complex patients. Methods. A prospective audit was performed of eight patients; three males and five females, aged 20–38 at diagnosis. Two patients commenced cinacalcet as primary treatment and six had previous surgery. Six patients had complications of hyperparathyroidism: renal calculi, renal dysfunction, and reduced bone mineral density. All were commenced on cinacalcet 30 mg bd for MEN1 associated hyperparathyroidism; doses were subsequently reduced to 30 mg od in four patients. Results. Significant reductions were observed in serum calcium and PTH measurements. Serum calcium reduced by a median of 0.35 mmol/L (=.012 Wilcoxon Signed Rank. Serum PTH levels decreased by a median of 5.05 pmol/L (=.012. There was no change in urine calcium. Duration ranged from 10–35 months with maintenance of control. Cinacalcet was well tolerated by six patients; one experienced nausea and one experienced diarrhoea. Conclusion. Cinacalcet is an effective and well-tolerated medical treatment for the management of complex primary hyperparathyroidism.

  8. Clinical Use of Cinacalcet in MEN1 Hyperparathyroidism

    Science.gov (United States)

    Moyes, V. J.; Monson, J. P.; Chew, S. L.; Akker, S. A.

    2010-01-01

    Background. Management of multiple-endocrine neoplasia type 1- (MEN1-) associated hyperparathyroidism is associated with high recurrence rates and high surgical morbidity due to multiple neck explorations. Cinacalcet, a calcimimetic agent licensed for the treatment of secondary hyperparathyroidism and parathyroid carcinoma, may provide a medical alternative for the management of these complex patients. Methods. A prospective audit was performed of eight patients; three males and five females, aged 20–38 at diagnosis. Two patients commenced cinacalcet as primary treatment and six had previous surgery. Six patients had complications of hyperparathyroidism: renal calculi, renal dysfunction, and reduced bone mineral density. All were commenced on cinacalcet 30 mg bd for MEN1 associated hyperparathyroidism; doses were subsequently reduced to 30 mg od in four patients. Results. Significant reductions were observed in serum calcium and PTH measurements. Serum calcium reduced by a median of 0.35 mmol/L (P = .012 Wilcoxon Signed Rank). Serum PTH levels decreased by a median of 5.05 pmol/L (P = .012). There was no change in urine calcium. Duration ranged from 10–35 months with maintenance of control. Cinacalcet was well tolerated by six patients; one experienced nausea and one experienced diarrhoea. Conclusion. Cinacalcet is an effective and well-tolerated medical treatment for the management of complex primary hyperparathyroidism. PMID:20585352

  9. Primary hyperparathyroidism due to an intrathyroidal parathyroid adenoma associated with chronic lymphocytic thyroiditis.

    Science.gov (United States)

    Cating-Cabral, Monica Therese; Cabungcal, Arsenio Claro; Villafuerte, Cesar Vincent; Añel-Quimpo, Joselynna

    2012-06-08

    This is a case of a 44-year-old woman with an anterior neck mass and hypothyroidism who presented with an incidental finding of an elevated serum calcium level and was found to have primary hyperparathyroidism and osteoporosis. During surgical exploration no parathyroid adenoma was found, although a nodule was palpated within the right thyroid lobe. Examination of the excised right thyroid lobe revealed an intrathyroidal parathyroid adenoma and chronic lymphocytic thyroiditis. After surgery, she did not develop severe hypocalcaemia and this was attributed to preoperative treatment with pamidronate. In the months following surgery, parathyroid hormone remained undetectable.

  10. Hyperparathyroidism-related extensor tenosynovitis at the wrist: a general review of the literature.

    Science.gov (United States)

    Ichihara, Satoshi; Hidalgo-Diaz, Juan Jose; Prunières, Guillaume; Facca, Sybille; Bodin, Frédéric; Boucher, Stéphanie; Liverneaux, Philippe

    2015-07-01

    Extensor tenosynovitis often occurs accompanying with rheumatoid arthritis, gout, trauma, mycobacterium and dialysis-related amyloidosis. However, there is no recognition of extensor tenosynovitis accompanying with hyperparathyroidism. The purpose of this general review was to describe the clinical condition and to report the results of surgical intervention in the extensor tenosynovitis at the wrist related to hyperparathyroidism. Hyperparathyroidism is thought to be a rare disease in adult. Although renal symptoms are the commonest symptom, musculoskeletal complaints also occur in hyperparathyroidism. From our general review, hyperparathyroidism deserves consideration in the differential diagnosis of extensor tenosynovitis at the wrist.

  11. Primary hyperparathyroidism: A changing scenario in India

    Science.gov (United States)

    Jha, Sangeeta; Jayaraman, Muthukrishnan; Jha, Aman; Jha, Ratan; Modi, Kirtikumar D.; Kelwadee, Jayant V.

    2016-01-01

    Introduction: Primary hyperparathyroidism (PHPT) is largely a symptomatic disease with varied systemic manifestations, complicated by coexisting Vitamin D (Vit D) deficiency. Increasing awareness, developments in diagnostics, and Vit D supplementation may have an impact on the disease profile of PHPT. Methods: Clinical, biochemical, and pathological profile of PHPT presenting to a tertiary care center in South India were compared in two groups separated as per the period of presentation (Group A: January 1994–May 2007 - 51 cases and Group B: June 2007–January 2015 - 59 cases). Results: PHPT has remained a disease of female preponderance with similar age of presentation. It is being diagnosed earlier (mean duration of symptoms prior to diagnosis was 38.7 months in Group A, significantly longer than 26 months in Group B). Bone pain and metabolic myopathy were the most common presentations (60%) followed by pathological fracture (16%), renal calculi (13%), and pancreatitis (7%). Pathological fractures have become less frequent. Vit D deficiency is still a widespread co-morbidity. Radionuclide scintigraphy is an effective localizing tool, but ultrasound can be an inexpensive and widely available screening modality. Conclusion: PHPT still remains asymptomatic disease of bones and stones, although it is being diagnosed early. Greater awareness, Vit D supplementation, and better diagnostic tools have made it a disease with lesser morbidity and effective cure. PMID:26904473

  12. Management of secondary hyperparathyroidism: how and why?

    Science.gov (United States)

    Komaba, Hirotaka; Kakuta, Takatoshi; Fukagawa, Masafumi

    2017-03-01

    Secondary hyperparathyroidism (SHPT) is a common complication in chronic kidney disease. Currently, various treatment options are available, including vitamin D receptor activators, cinacalcet hydrochloride, and parathyroidectomy. These treatment options have contributed to the successful control of SHPT, and recent clinical studies have provided evidence suggesting that effective treatment of SHPT leads to improved survival. Although bone disease is the most widely recognized consequence of SHPT and remains a major target for treatment of SHPT, there is increasing evidence that parathyroid hormone (PTH) and fibroblast growth factor 23 (FGF23), both of which are markedly elevated in SHPT, have multiple adverse effects on extraskeletal tissues. These actions may lead to the pathological development of left ventricular hypertrophy, renal anemia, immune dysfunction, inflammation, wasting, muscle atrophy, and urate accumulation. Given that treatment of SHPT leads to decreases in both PTH and FGF23, these data provide an additional rationale for treating SHPT. However, definitive evidence is still lacking, and future research should focus on whether treatment of SHPT prevents the adverse effects of PTH and FGF23.

  13. Primary hyperparathyroidism at nishi kobe medical center

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    Omori, Koichi; Yamashita, Masaru; Kishimoto, Masanao [Nishi Kobe Medical Center, Hyogo (Japan); Tsuji, Tomoko [Kyoto Univ. (Japan). Hospital

    2002-03-01

    Since 1994, there have been 10 cases (1 male, 9 females, 37-72 years old) of primary hyperparathyroidism at our hospital. CT, ultrasonography (US), and MIBI scintigraphy (MIBI) were undertaken for preoperative imaging of the parathyroid tumor. In 9 adenoma cases, accurate localization was obtained in 6 of 9 cases for US, in 8 of 8 cases for CT, and in 9 of 9 cases for MIBI. After the surgery, laboratory data of Ca and i-PTH were in the normal range. Histopathological diagnosis showed 9 adenoma cases and 1 hyperplasia case. Three cases were associated with thyroid diseases. This paper presents two cases with rare clinical symptoms. The chief complaint of the first case was hoarseness. Transient recurrent nerve palsy was observed two times in 2 years. During the surgery, the tumor existed just behind the right recurrent nerve. By histological examination, the tumor was diagnosed as parathyroid adenoma. There has been no recurrent nerve palsy since the surgery. The second case had been treated after diagnosis of deglusion for 3 years in a psychiatry clinic. CT and MIBI showed parathyroid tumor just below the right lobe of the thyroid. After surgery, psychological symptoms were dramatically improved. (author)

  14. A novel mitochondrial DNA deletion in a patient with Pearson syndrome and neonatal diabetes mellitus provides insight into disease etiology, severity and progression.

    Science.gov (United States)

    Chen, Xin-Yu; Zhao, Si-Yu; Wang, Yan; Wang, Dong; Dong, Chang-Hu; Yang, Ying; Wang, Zhi-Hua; Wu, Yuan-Ming

    2016-07-01

    Pearson syndrome (PS) is a rare, mitochondrial DNA (mtDNA) deletion disorder mainly affecting hematopoietic system and exocrine pancreas in early infancy, which is characterized by multi-organ involvement, variable manifestations and poor prognosis. Since the clinical complexity and uncertain outcome of PS, the ability to early diagnose and anticipate disease progression is of great clinical importance. We described a patient with severe anemia and hyperglycinemia at birth was diagnosed with neonatal diabetes mellitus, and later with PS. Genetic testing revealed that a novel mtDNA deletion existed in various non-invasive tissues from the patient. The disease course was monitored by mtDNA deletion heteroplasmy and mtDNA/nucleus DNA genome ratio in different tissues and at different time points, showing a potential genotype-phenotype correlation. Our findings suggest that for patient suspected for PS, it may be therapeutically important to first perform detailed mtDNA analysis on non-invasive tissues at the initial diagnosis and during disease progression.

  15. Mild hypoxemia during initial reperfusion alleviates the severity of secondary energy failure and protects brain in neonatal mice with hypoxic-ischemic injury.

    Science.gov (United States)

    Niatsetskaya, Zoya V; Charlagorla, Pradeep; Matsukevich, Dzmitry A; Sosunov, Sergey A; Mayurasakorn, Korapat; Ratner, Veniamin I; Polin, Richard A; Starkov, Anatoly A; Ten, Vadim S

    2012-02-01

    Reperfusion triggers an oxidative stress. We hypothesized that mild hypoxemia in reperfusion attenuates oxidative brain injury following hypoxia-ischemia (HI). In neonatal HI-mice, the reperfusion was initiated by reoxygenation with room air (RA) followed by the exposure to 100%, 21%, 18%, 15% oxygen for 60 minutes. Systemic oxygen saturation (SaO(2)), cerebral blood flow (CBF), brain mitochondrial respiration and permeability transition pore (mPTP) opening, markers of oxidative injury, and cerebral infarcts were assessed. Compared with RA-littermates, HI-mice exposed to 18% oxygen exhibited significantly decreased infarct volume, oxidative injury in the brain mitochondria and tissue. This was coupled with improved mitochondrial tolerance to mPTP opening. Oxygen saturation maintained during reperfusion at 85% to 95% was associated (r=0.57) with the best neurologic outcome. Exposure to 100% or 15% oxygen significantly exacerbated brain injury and oxidative stress. Compared with RA-mice, hyperoxia dramatically increased reperfusion CBF, but exposure to 15% oxygen significantly reduced CBF to values observed during the HI-insult. Mild hypoxemia during initial reperfusion alleviates the severity of HI-brain injury by limiting the reperfusion-driven oxidative stress to the mitochondria and mPTP opening. This suggests that at the initial stage of reperfusion, a slightly decreased systemic oxygenation (SaO(2) 85% to 95%) may be beneficial for infants with birth asphyxia.

  16. Systematic review of oral manifestations related to hyperparathyroidism.

    Science.gov (United States)

    Palla, Benjamin; Burian, Egon; Fliefel, Riham; Otto, Sven

    2017-06-14

    We sought to identify oral symptoms found in hyperparathyroidism and compare their rate of occurrence, as well as potential variations in sequelae between primary, secondary, and tertiary hyperparathyroidism. Database searches were performed through EMBASE and PubMed, with a continual handsearch for relevant articles. PRISMA guidelines were followed. Two hundred five articles including 245 patients were analyzed with data extraction. The average age was 34.02 years old (age range 1-83), with 91 male and 154 female patients (1:1.7 M/F ratio). Patients presented with symptoms including facial asymmetry or swelling (167/214 cases; 78.0%), oral pain (30/214; 14.0%), systemic symptoms (25/214; 11.7%), referrals or incidental findings (16/214; 7.5%), and neuropathy (6/214; 2.8%) independently and in combination together. Bony pathology occurred most often in the mandible (100/245 cases; 40.8%), while 72 cases were in the maxilla (29.4%) and 73 cases in both jaw bones (29.8%). Our data collection identifies a wide variation in the presentation of hyperparathyroidism. In order to be more certain of oral maladies from hyperparathyroidism, studies with large patient populations need to be conducted at healthcare centers to clarify the oral outcomes of hyperparathyroidism. What was thought to be a characteristic finding of HPT, mandibular radiolucency occurred in only a minor portion of cases. Furthermore, the pathognomonic sign of HPT on radiograph, loss of lamina dura, was only the third most common presentation. Bone pathology was most commonly reported in literature, but should not be assumed the only oral sequelae of hyperparathyroidism.

  17. Lipoadenoma of the parathyroid gland - a rare cause of hyperparathyroidism.

    Science.gov (United States)

    Bansal, R; Trivedi, P; Sarin, J; Parikh, B

    2012-01-01

    Lipoadenoma of parathyroid gland is an unusual morphologic variant of parathyroid adenoma in which the glandular elements are associated with abundant mature adipose tissue. The lesion has also been reported as parathyroid lipohyperplasia, parathyroid hamartoma, and parathyroid adenoma with myxoid stroma. Most cases are functioning and are associated with hyperparathyroidism. Lipoadenoma of parathyroid gland are difficult to diagnose as a cause of hyperparathyroidism because of rarity of these lesions and overlap with normal parathyroid tissue on microscopic evaluation. Only few cases have been documented in the literature so far. The lesion may be overlooked by both surgeon and pathologists alike, if they are not aware of this specific clinicopathologic entity.

  18. Imaging for primary hyperparathyroidism -- what beginners should know

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    Ahuja, A.T. E-mail: aniltahuja@cuhk.edu.hk; Wong, K.T.; Ching, A.S.C.; Fung, M.K.; Lau, J.Y.W.; Yuen, E.H.Y.; King, A.D

    2004-11-01

    For patients with primary hyperparathyroidism surgical removal of the hyperfunctioning parathyroid gland is curative. With advances in minimally invasive surgery, accurate pre-operative localization of the hyperfunctioning parathyroid tissue is essential to aid successful surgical treatment. The onus of identifying this hyperfunctioning parathyroid tissue therefore falls on imaging techniques such as high-resolution ultrasound, radionuclide imaging, computed tomography and magnetic resonance imaging. This article is not an exhaustive review, and its main aim is to familiarize the general radiologist, trainee radiologists and clinicians with the basics of various imaging techniques and their roles in practical management of patients with primary hyperparathyroidism.

  19. Primary hyperparathyroidism: epidemiology, clinical features, diagnostic tools and current management

    Directory of Open Access Journals (Sweden)

    Andrea Percivale

    2015-12-01

    Full Text Available Primary hyperparathyroidism (PHPT is a clinical condition characterized by overactive parathyroid gland secretion of parathyroid hormone with concurrent alteration of the phosphocalcemic metabolism. We present a literature review on primary hyperparathyroidism addressing key on clinical presentation, causes, medical and surgical treatment at the best of our knowledge. Based on this review we confirm the role of serum calcium and serum level examination, as well as we define the definitive treatment for PHPT being parathyroidectomy. In case of contraindication for surgery, medical treatment can play a relevant role.

  20. Cinacalcet Effectively Reduces Parathyroid Hormone Secretion and Gland Volume Regardless of Pretreatment Gland Size in Patients with Secondary Hyperparathyroidism

    Science.gov (United States)

    Komaba, Hirotaka; Nakanishi, Shohei; Fujimori, Akira; Tanaka, Motoko; Shin, Jeongsoo; Shibuya, Koji; Nishioka, Masato; Hasegawa, Hirohito; Kurosawa, Takeshi

    2010-01-01

    Background and objectives: Cinacalcet is effective in reducing serum parathyroid hormone (PTH) in patients with secondary hyperparathyroidism. However, it has not been proven whether parathyroid gland size predicts response to therapy and whether cinacalcet is capable of inducing a reduction in parathyroid volume. Design, setting, participants, & measurements: This 52-week, multicenter, open-label study enrolled hemodialysis patients with moderate to severe secondary hyperparathyroidism (intact PTH >300 pg/ml). Doses of cinacalcet were adjusted between 25 and 100 mg to achieve intact PTH 30% reduction from baseline (88 versus 78%), but this was not statistically significant. Cinacalcet therapy also resulted in a significant reduction in parathyroid gland volume regardless of pretreatment size, which was in sharp contrast to historical controls (n = 87) where parathyroid gland volume progressively increased with traditional therapy alone. Conclusions: Cinacalcet effectively decreases serum PTH levels and concomitantly reduces parathyroid gland volume, even in patients with marked parathyroid hyperplasia. PMID:20798251

  1. Is Primary Hyperparathyroidism a Risk Factor for Papillary Thyroid Cancer? An Exemplar Study and Literature Review

    Directory of Open Access Journals (Sweden)

    Cinamon, Udi

    2014-12-01

    Full Text Available Introduction Primary hyperparathyroidism (PHPT is associated with several cancer types, including papillary thyroid carcinoma (PTC. Objective To explore further the relation between PHPT and PTC. Methods By considering patients with PHPT as extra-suspicious for PTC, we studied an exemplar group of patients with PHPT with a small (≤1 cm thyroid nodule, which was negative in preoperative cytologic examination. During parathyroidectomy, a frozen section biopsy of the thyroid nodule confirmed PTC, as did the final surgical specimen, revealing that the preoperative cytology was false-negative. Additionally, relevant reports retrieved from the English literature addressing thyroid cancer and hyperparathyroidism were reviewed and processed. Results Four patients with PHPT were studied. Three had a multifocal thyroid disease, and three had neck lymph node metastasis. Processing previous report data supported an association between PHPT and PTC. Although thyroid nodularity among patients with PHPT was similar to the general population, PTC incidence was higher. This was true also for patients with secondary hyperparathyroidism. Conclusions This study emphasized that PHPT should be considered as a noteworthy risk factor for PTC. Fine needle aspiration of a thyroid nodule is the most valuable diagnostic procedure for thyroid cancer. Yet, false-negative results were reported in up to 10% of cases, especially in small, subcentimeter nodules. In line with our data and the literature, patients with PHPT should have both a detailed ultrasound addressing the thyroid and cytology of any thyroid nodule, including small subcentimeter lesions. Moreover, surgical flexibility, allowing intraoperative thyroid nodule sampling, should be considered even for “innocent” nodules.

  2. Hyperparathyroidism as a cause of recurrent acute pancreatitis: A case report

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    Tešić-Rajković Snežana

    2016-01-01

    Full Text Available Introduction. One of the more uncommon etiological factors responsible for the development of acute pancreatitis (AP is hypercalcemia. Hyperparathyroidism (HPT, as a cause of hypercalcemia, is responsible for 1.5–13% of AP according to a number of studies. A mechanism of the development of AP in hyperparathyroidism is still unclear. Case report. We presented a 47-year-old female patient, who had five episodes of AP in total before the etiological factors were finally determined. The patient had certain comorbidities which were considered to be potential causes of AP. She had chronic renal insufficiency (she was on a regular hemodialysis program, systemic lupus erythematosus and mioma uteri. She used to regularly take an antiepileptic drug (combination of sodium valproate and valproic acid. During the fifth episode of AP, the serum calcium level was for the first time elevated to twice the normal value. Level of parathyroid hormone was several times higher. A static scintigraphy found hyperplasia or hyperfunctional adenoma of the right inferior and superior parathyroid glands. Abdominal multislice computed tomography (MSCT scan verified the enlargement of the entire pancreas, as well as the presence of heterogeneous structures with diffuse amorphous calcifications. The lytic lesions in the pelvic bones could be seen in both sides. Parathyroidectomy was being postponed by an endocrine surgeon because of the poor overall condition of the patient. In the next period the patient had five more episodes of AP. The condition was significantly contributed by increasingly more frequent and longer episodes of metrorrhagia. Despite all therapeutic measures that were taken, systemic inflammatory response syndrome (SIRS developed, and fatal outcome occurred. Conclusion. In case of recurrent pancreatitis, hyperparathyroidism is to be considered even if a significant elevation of serum calcium is not present. This is especially the case for patients with

  3. Diagnosis of hyperparathyroidism; Diagnostik des Hyperparathyreoidismus

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    Delorme, S. [Abteilung fuer Onkologische Diagnostik und Therapie, Deutsches Krebsforschungszentrum, Heidelberg (Germany); Hoffner, S. [Abteilung Nuklearmedizin, Universitaetsklinik Heidelberg (Germany)

    2003-04-01

    To preoperatively localize enlarged parathyroid glands in patients with hyperparathyroidism (HPT). Besides clinical and biochemical workup, high-resolution ultrasonography (US) is the most commonly used imaging method.Additionally,Tc-99m-MIBI scintigraphy in subtraction or biphasic technique, computed tomography (CT) and magnetic resonance imaging (MRI) are used. US fails to detect a minimum of 10% of enlarged parathyroid glands,most commonly due to ectopic location, or difficult examination conditions, such as nodular goiter or previous surgery. If attempted US localization is unsuccessful, multiphase scintigraphy, using Tc-99m-sestamibi, can help to locate ectopic adenomas in the mediastinum. With SPECT, ademomas can be found which escape detection on planar scans due to their small size.With combined use of US and scintigraphy, a correct localization of parathyroid adenomas is possible in up to 90%. CT and MRI are of limited value due to their low specificity. For newly diagnosed hyperparathyroidism, high-resolution US is the method of choice for localizing parathyroid adenomas.If ultrasound fails to detect a lesion, Tc-99m-MIBI scintigraphy is recommended. In patients scheduled for re-operation for recurrent or persistant HPT, a preoperative detection of a parathyroid adenoma should be attempted whenever possible, in order to minimize the extent of surgery. The role of CT or MRI is mainly to help to better anatomically localize a suspected adenoma previously detected with scintigraphy. (orig.) [German] Praeoperative Lokalisation vergroesserter Epithelkoerperchen bei Patienten mit primaerem Hyperparathyreoidismus (HPT).Methoden Neben klinischem Befund und laborchemischer Abklaerung ist der hochaufloesende Ultraschall die am haeufigsten eingesetzte Methode.Zusaetzliche Methoden sind Tc-99m-MIBI-Szintigraphie in Subtraktions- oder Zweiphasentechnik,Computertomographie (CT) oder Magnetresonanztomographie (MRT).Ergebnisse Mit der Sonographie gelingt eine

  4. Ultrasound in clinical setting of secondary hyperparathyroidism.

    Science.gov (United States)

    Meola, Mario; Petrucci, Ilaria; Cupisti, Adamasco

    2013-01-01

    Secondary hyperparathyroidism (sHPT) is one of the most common and serious complications of chronic kidney disease (CKD) and maintenance hemodialysis (MHD). In sHPT, the biology of parathyroid cells changes significantly toward diffuse and nodular hyperplasia. Diagnosis and treatment of sHPT are based on intact parathyroid hormone (i-PTH) serum levels and on the parameters of mineral metabolism. The morphological diagnosis of sHPT relies on 2 complementary imaging techniques: high-resolution ultrasonography with color Doppler imaging (US/CD) and 99mTc-methoxyisobutylisonitrile (MIBI) scintigraphy. The main objective of this review is to stimulate nephrologists to use US/CD of the parathyroid glands during the progression of CKD in order to aid clinical, pharmacological and surgical strategies. The primary role of US/CD in sHPT should be to integrate the clinical diagnosis by defining the number and volume of hyperplastic glands, although the international guidelines do not state when and why to perform US/CD. This review also evaluates the role of US/CD in clinical follow-up and assessment of therapeutic response of sHPT, and it highlights how US/CD can evaluate the effect of therapy with phosphate binders, vitamin D or its analogues and calcimimetics, which are changing the natural history of sHPT and the frequency of parathyroidectomy. Evaluation of the morphological and vascular changes of hyperplastic parathyroids is useful to guide percutaneous ethanol injection therapy and to support clinical, pharmacological and surgical strategies. Epidemiological studies are needed to establish how US/CD could change the management of sHPT and why it should be repeated in patients with high levels of serum i-PTH.

  5. A Rare Electrocardiographic Manifestation of A Rare form of Multiple Electrolyte Disturbances: Hyperparathyroid Crisis

    Directory of Open Access Journals (Sweden)

    Mitra Chitsazan

    2011-12-01

    Full Text Available The surface electrocardiogram (ECG has been used as a useful method for detection of metabolic disturbances for a long time. However, it may be difficult to distinguish the exact disturbance when more than one metabolic abnormality exists in a patient simultaneously. Although, "classic" ECG characterizations of common electrolyte disturbances are well described, multiple concurrent electrolyte disturbances may lead to ECG abnormalities that may not be easily detectable. This ECG concerns a 60-year-old male presented with general fatigue, weakness, epigastric pain, anorexia, nausea and extreme hypercalcemia (serum total and ionized calcium levels 20.5 mg/dL and 12.02 mg/dl, respectively, hypokalemia and hypomagnesemia associated with elevated parathyroid hormone (1160 pg/ml and normal serum vitamin D level (97 ng/ml . This rare manifestation of primary hyperparathyroidism has been named hyperparathyroid crisis in the literature. Hyperparathyroid crisis is an emergency form of multiple electrolyte abnormalities that manifest as a life-threatening hypercalcemia and simultaneous hypokalemia and hypomagnesemia; these two later are believed to be caused by diuretic effect of calcium on the renal tubules. The unique pattern of ECG in our patient first was misdiagnosed as prominent T waves with prolongation of the QT corrected (QTc interval, which has been reported several times in patients with hyperparathyroidism crisis, compatible with our patient. But more investigation revealed that, the QTc interval not only is not prolonged, it is shortened as it is expected from the effect of hypercalcemia on electrocardiogram. The exact pattern of the patient`s ECG (figure 1 can be interpreted as it follows: (1 Flattening of the T wave, (2 a prominent U wave, (3 prolongation of the descending limb of the T wave such that it overlapped with the next U wave (4 virtual absence of ST segment and (5 shortening of the QT corrected interval. In conclusion, it should

  6. pPKCα mediated-HIF-1α activation related to the morphological modifications occurring in neonatal myocardial tissue in response to severe and mild hyperoxia

    Directory of Open Access Journals (Sweden)

    S. Zara

    2012-01-01

    Full Text Available In premature babies birth an high oxygen level exposure can occur and newborn hyperoxia exposure can be associated with free radical oxygen release with impairment of myocardial function, while in adult animal models short exposure to hyperoxia seems to protect heart against ischemic injury. Thus, the mechanisms and consequences which take place after hyperoxia exposure are different and related to animals age. The aim of our work has been to analyze the role played by HIF-1α in the occurrence of the morphological modifications upon hyperoxia exposure in neonatal rat heart. Hyperoxia exposure induces connective compartment increase which seems to allow enhanced blood vessels growth. An increased hypoxia inducible factor-1α (HIF-1α translocation and vascular endothelial growth factor (VEGF expression has been found upon 95% oxygen exposure to induce morphological modifications. Upstream pPKC-α expression increase in newborn rats exposed to 95% oxygen can suggest PKC involvement in HIF-1α activation. Since nitric oxide synthase (NOS are involved in heart vascular regulation, endothelial NOS (e-NOS and inducible NOS (i-NOS expression has been investigated: a lower eNOS and an higher iNOS expression has been found in newborn rats exposed to 95% oxygen related to the evidence that hyperoxia provokes a systemic vasoconstriction and to the iNOS pro-apoptotic action, respectively. The occurrence of apoptotic events, evaluated by TUNEL and Bax expression analyses, seems more evident in sample exposed to severe hyperoxia. All in all such results suggest that in newborn rats hyperoxia can trigger oxygen free radical mediated membrane injury through a pPKCα mediated HIF-1α signalling system, even though specificity of such response could be obtained by in vivo administration to the rats of specific inhibitors of PKCα. This intracellular signalling can switch molecular events leading to blood vessels development in parallel to pro-apoptotic events

  7. Anaesthetic management of emergency pacemaker implantation in a case of neonatal lupus erythematosus with complete congenital heart block & severe respiratory distress

    Directory of Open Access Journals (Sweden)

    Usha Kiran

    2007-01-01

    Full Text Available An 8-week old 3-kilogram male baby was brought to this tertiary care hospital with respiratory distress, marked tracheal tug, poor feeding and a heart rate of 46/minute. The child had been referred from a peripheral hospital as a case of neonatal lupus with complete congenital heart block. The mother was seropositive for systemic lupus erythematosus with a history of two abortions. Evaluation on admission revealed a heart rate between 40-60/ minute, respiratory rate 40-50/ minute, inspiratory stridor, bilateral crepitations, chest retrac-tion and a marked tracheal tug that improved with prone positioning. Electrocardiography and echocardiography confirmed complete congenital heart block with cardiomegaly and mild left ventricular dysfunction. Keeping in view the impending congestive heart failure, possible early cardiomyopathy and the bad obstetric history ur-gent pacemaker implantation was planned to allow early recovery of the child. The anaesthetic risk was high due to the heart block, ventricular dysfunction, laryngomalacia, severe tracheal tug and anticipated difficult weaning from controlled ventilation. General anaesthesia was administered with endotracheal tube and con-trolled ventilation using ketamine, rocuronium and sufentanil. For patient safety invasive monitoring was pro-vided and external pacing was kept standby. Epicardial pacemaker leads were implanted onto the left ventricu-lar wall through a left anterior 6th intercostal space thoracotomy. The child was electively ventilated for two post operative days. The tracheal tug and secretions gradually subsided over 2 weeks with oxygen, antibiotics, steroids, bronchodilators and physiotherapy. At the time of discharge from hospital 2 weeks after the implant the child was feeding well, tracheal tug was minimal and the lungs were clear.

  8. ERYTHEMA NODOSUM AND PROLONGED FEVER ASSOCIATED TO SECONDARY HYPERPARATHYROIDISM

    Directory of Open Access Journals (Sweden)

    Galimberti R

    2005-08-01

    Full Text Available SUMMARYSecondary hyperparathyroidism is one of the main deragements caused by chronic renal failure, and parathyroid hormone is considered one of the toxins of the uremic syndrome. Prolonged fever due to primary hyperparathyroidism have already been described in the literature but not yet as induced by secondary hyperparathyroidism. In this case report a patient suffering from an erythema nodosum and prolonged fever associated to secondary hyperparathyroidism that disappeared through subtotal parathyroidectomy is presented.RESUMENEl hiperparatiroidismo secundario es uno de los principales disturbios causados por la insuficiencia renal crónica, y la paratohormona es considerada una de las toxinas del sindrome urémico. El sindrome febril prolongado secundario a hiperparatiroidismo primario ya ha sido descripto en la literatura, aunque no lo ha sido aun el inducido por hiperparatiroidismo secundario. En el presente reporte se presenta un caso de eritema nodoso y sindrome febril prolongado asociado a hiperparatiroidismo secundario y que resolvió luego de efectuada una paratiroidectomía subtotal.

  9. Surgical treatment of hyperparathyroidism : with an analysis of 267 cases

    NARCIS (Netherlands)

    H.A. Bruining (Hajo)

    1971-01-01

    textabstractIt is generally accepted that for autonomous hyperparathyroidism, whether primary or tertiary, surgery is still the only suitable method of treatment available. Analysis of a series of cases treated in t his way over the past twenty years has shown that there are certain problems associa

  10. Vitamin D metabolites and skeletal consequences in primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Moosgaard, Bjarke; Christensen, Signe Engkjær; Vestergaard, Peter

    2008-01-01

    BACKGROUND: Primary hyperparathyroidism (PHPT) is associated with reduced bone mineral density (BMD) mainly at sites rich in cortical bone. However, successful parathyroidectomy causes an increase in BMD especially at sites rich in trabecular bone. Plasma 25-hydroxyvitamin D (25OHD) levels...

  11. Recurrent primary hyperparathyroidism due to Type 1 parathyromatosis.

    Science.gov (United States)

    Jain, Monica; Krasne, David L; Singer, Frederick R; Giuliano, Armando E

    2017-02-01

    Parathyromatosis is a rare condition consisting of multiple nodules of benign hyperfunctioning parathyroid tissue scattered throughout the neck and superior mediastinum. As a potential cause of recurrent or persistent hyperparathyroidism, parathyromatosis is a challenging condition to diagnose and treat. The optimal evaluation and management of patients with parathyromatosis is not well established. The reported case involves a patient who was initially diagnosed with primary hyperparathyroidism. The diagnosis of Type 1 parathyromatosis was made after the patient developed recurrent hyperparathyroidism with hypercalcemia and osteoporosis 17 years after the initial operation and underwent two additional operations. The majority of parathyromatosis cases are diagnosed in the setting of secondary hyperparathyroidism. Consensus regarding the preoperative diagnosis and evaluation is lacking due to the paucity of cases of this rare clinical entity. Management involves complete surgical extirpation of all identifiable rests of parathyroid tissue. Intra-operative parathyroid hormone level monitoring and frozen section examination are excellent tools that could increase the rates of initial operative success. Despite this, long-term disease remission is rare, and medical therapy, including calcimimetics and bisphosphonates, may be required for postoperative or non-operative management.

  12. Primary hyperparathyroidism with rare presentation as multiple brown tumours

    Directory of Open Access Journals (Sweden)

    Smit Doshi

    2012-04-01

    Full Text Available We present a case of primary hyperparathyroidism with an uncommon presentation as multiple brown tumours, which may easily be mistaken for a primary bone neoplasm. A brief literature review and its clinical and surgical management are also discussed here.

  13. Evaluation of secondary hyperparathyroidism in patients undergoing hemodialysis.

    Science.gov (United States)

    Rahimian, Mohammad; Sami, Ramin; Behzad, Fariba

    2008-01-01

    Renal osteodystrophy is a complication of chronic kidney disease (CKD) that present in low and high turnover patterns. This disorder has a key role in the disability of CKD patients in whom early diagnosis and treatment can result in better outcome. We studied hyperparathyroidism prevalence and its relationship with renal osteodystrophy in our advanced CKD population. We included 80 patients (of whom 44 (55%) were diabetic) during 6 months period. The patients answered a questionnaire about symptoms related to bone disease and blood levels of parathormone (PTH), calcium, phosphorus, and alkaline phosphatase were obtained, in addition to hand and skull radiographs in all the study patients. Prevalence of clinically evident hyperparathyroidism in our patients was 45%. Hyperparathyroidism had significant relationship with alkaline phosphatase and radiological findings, but did not have a significant relationship with dialysis duration, age, sex, familial history, diabetes mellitus, or hypertension. We conclude that secondary hyperparathyroidism is prevalent in our dialysis population and has high correlation with serum alkaline phosphatase levels and radiological changes.

  14. Monitoring neonates for ototoxicity.

    Science.gov (United States)

    Garinis, Angela C; Kemph, Alison; Tharpe, Anne Marie; Weitkamp, Joern-Hendrik; McEvoy, Cynthia; Steyger, Peter S

    2017-06-22

    Neonates admitted to the neonatal intensive care unit (NICU) are at greater risk of permanent hearing loss compared to infants in well mother and baby units. Several factors have been associated with this increased prevalence of hearing loss, including congenital infections (e.g. cytomegalovirus or syphilis), ototoxic drugs (such as aminoglycoside or glycopeptide antibiotics), low birth weight, hypoxia and length of stay. The aetiology of this increased prevalence of hearing loss remains poorly understood. Here we review current practice and discuss the feasibility of designing improved ototoxicity screening and monitoring protocols to better identify acquired, drug-induced hearing loss in NICU neonates. A review of published literature. We conclude that current audiological screening or monitoring protocols for neonates are not designed to adequately detect early onset of ototoxicity. This paper offers a detailed review of evidence-based research, and offers recommendations for developing and implementing an ototoxicity monitoring protocol for young infants, before and after discharge from the hospital.

  15. Vitamin D Metabolites and Their Association with Calcium, Phosphorus, and PTH Concentrations, Severity of Illness, and Mortality in Hospitalized Equine Neonates.

    Directory of Open Access Journals (Sweden)

    Ahmed M Kamr

    Full Text Available Hypocalcemia is a frequent abnormality that has been associated with disease severity and outcome in hospitalized foals. However, the pathogenesis of equine neonatal hypocalcemia is poorly understood. Hypovitaminosis D in critically ill people has been linked to hypocalcemia and mortality; however, information on vitamin D metabolites and their association with clinical findings and outcome in critically ill foals is lacking. The goal of this study was to determine the prevalence of vitamin D deficiency (hypovitaminosis D and its association with serum calcium, phosphorus, and parathyroid hormone (PTH concentrations, disease severity, and mortality in hospitalized newborn foals.One hundred newborn foals ≤72 hours old divided into hospitalized (n = 83; 59 septic, 24 sick non-septic [SNS] and healthy (n = 17 groups were included. Blood samples were collected on admission to measure serum 25-hydroxyvitamin D3 [25(OHD3], 1,25-dihydroxyvitamin D3 [1,25(OH 2D3], and PTH concentrations. Data were analyzed by nonparametric methods and univariate logistic regression. The prevalence of hypovitaminosis D [defined as 25(OHD3 <9.51 ng/mL] was 63% for hospitalized, 64% for septic, and 63% for SNS foals. Serum 25(OHD3 and 1,25(OH 2D3 concentrations were significantly lower in septic and SNS compared to healthy foals (P<0.0001; P = 0.037. Septic foals had significantly lower calcium and higher phosphorus and PTH concentrations than healthy and SNS foals (P<0.05. In hospitalized and septic foals, low 1,25(OH2D3 concentrations were associated with increased PTH but not with calcium or phosphorus concentrations. Septic foals with 25(OHD3 <9.51 ng/mL and 1,25(OH 2D3 <7.09 pmol/L were more likely to die (OR=3.62; 95% CI = 1.1-12.40; OR = 5.41; 95% CI = 1.19-24.52, respectively.Low 25(OHD3 and 1,25(OH2D3 concentrations are associated with disease severity and mortality in hospitalized foals. Vitamin D deficiency may contribute to a pro-inflammatory state in equine

  16. Neonatal anemia.

    Science.gov (United States)

    Aher, Sanjay; Malwatkar, Kedar; Kadam, Sandeep

    2008-08-01

    Neonatal anemia and the need for red blood cell (RBC) transfusions are very common in neonatal intensive care units. Neonatal anemia can be due to blood loss, decreased RBC production, or increased destruction of erythrocytes. Physiologic anemia of the newborn and anemia of prematurity are the two most common causes of anemia in neonates. Phlebotomy losses result in much of the anemia seen in extremely low birthweight infants (ELBW). Accepting a lower threshold level for transfusion in ELBW infants can prevent these infants being exposed to multiple donors.

  17. Primary hyperparathyroidism masquerading as rickets: diagnostic challenge and treatment outcomes.

    Science.gov (United States)

    Dutta, Deep; Kumar, Manoj; Das, Ram Narayan; Datta, Saumik; Biswas, Dibakar; Ghosh, Sujoy; Mukhopadhyay, Satinath; Chowdhury, Subhankar

    2013-01-01

    Primary hyperparathyroidism (PHPT) is extremely uncommon among children and is more likely to be associated with genetic syndromes, multiglandular involvement, and more severe symptoms. Rickets can very rarely be the presenting feature of PHPT in children. Rickets was diagnosed in a 12-year-old girl presenting with short stature, genu valgum, eversion deformity at the ankle joints, and flat feet. Radiograms showed generalized osteopenia, widening of the distal ends of the long bones along with splaying, cupping and fraying. Biochemical evaluation revealed low serum calcium (7.8 mg/dL), low phosphorus (1.4 mg/dL), vitamin-D deficiency [25-hydroxy-vitamin-D (25(OH)D): 8.7 ng/mL], and elevated intact parathyroid hormone (PTH, 811 pg/mL). Re-evaluation due to lack of clinical improvement following vitamin-D and calcium supplementation revealed hypercalcemia 11.9 mg/dL, normal 25(OH)D 41 ng/mL, persistence of elevated PTH 632 pg/mL. A 99mTc-sestamibi scan showed increased uptake at the lower pole of the right lobe of the thyroid. A right inferior parathyroidectomy was performed. Histopathology revealed chief cell type parathyroid adenoma. Last evaluated 4 months after surgery, the bone pains and proximal weakness had resolved, with significant improvement in the patient's quality of life. Rickets in the setting of PHPT often masks the classical phenotype of PHPT. In a child with rickets, lack of improvement following vitamin-D supplementation, hypercalcemia at presentation or following vitamin-D supplementation are warning signs which necessitate further evaluation to rule out PHPT.

  18. A novel surgical strategy for secondary hyperparathyroidism: Purge parathyroidectomy.

    Science.gov (United States)

    Shan, Cheng-Xiang; Qiu, Nian-Cun; Zha, Si-Luo; Liu, Miao-E; Wang, Qiang; Zhu, Pei-Pei; Du, Zhi-Peng; Xia, Chun-Yan; Qiu, Ming; Zhang, Wei

    2017-07-01

    This study was intended to demonstrate the feasibility and efficacy of purge parathyroidectomy (PPTX) for patients with secondary hyperparathyroidism (SHPT). The "seed, environment, and soil" medical hypothesis was first raised, following review of the literatures, to demonstrate the possible causes of persistence or recurrence of SHPT after parathyroidectomy. Subsequently, the novel surgical strategy of PPTX was proposed, which involves comprehensive resection of the fibro-fatty tissues, including visible or invisible parathyroid, within the region surrounded by the thyroid cartilage, bilateral carotid artery sheath, and the brachiocephalic artery. The perioperative information and clinical outcomes of patients who underwent PPTX from June 2016 to December 2016 were analyzed. In total, PPTX was performed safely in nine patients with SHPT from June 2016 to December 2016. The operative time for PPTX ranged from 95 to 135 min, and blood loss ranged from 20 to 40 mL. No patients with perioperative death, bleeding, convulsions, or recurrent laryngeal nerve injury were reported. The preoperative concentration of PTH ranged from 1062 to 2879 pg/mL, and from 12.35 to 72.69 pg/mL on the first day after surgery. In total, 37 parathyroid glands were resected. The postoperative pathologic examination showed that supernumerary or ectopic parathyroid tissues were found within the "non-parathyroid" tissues in three patients. No cases encountered persistence or recurrence of SHPT, or severe hypocalcemia during the follow-up period. PPTX involves comprehensive resection of supernumerary and ectopic parathyroid tissues, which may provide a more permanent means of reducing PTH levels. Copyright © 2017. Published by Elsevier Ltd.

  19. Circulating leptin and adiponectin levels in patients with primary hyperparathyroidism.

    Science.gov (United States)

    Delfini, Enrica; Petramala, Luigi; Caliumi, Chiara; Cotesta, Darlo; De Toma, Giorgio; Cavallaro, Giuseppe; Panzironi, Giuseppe; Diacinti, Daniele; Minisola, Savatore; D' Erasmo, Emilio; Mazzuoli, Gian Franco; Letizia, Claudio

    2007-01-01

    Primary hyperparathyroidism (PHPT) has been associated with high cardiovascular morbidity and mortality; its pathogenesis is not fully understood. Moreover, many metabolic abnormalities are frequently present in patients with PHPT. Several substances (such as leptin and adiponectin) are secreted from adipocytes, which may contribute to regulate energy homeostasis and the development of cardiovascular diseases. We examined the relationship between leptin and adiponectin levels and metabolic disorders in 67 newly diagnosed never-treated patients with PHPT and in 46 healthy subjects (HS). Twenty (29.8%) patients with PHPT presented a metabolic syndrome (as defined by Adult Treatment Panel III criteria). Serum leptin and adiponectin levels in HS were 6.28 +/- 3.3 ng/mL (range, 1.7-19.2 ng/mL) and 6.65 +/- 1.7 microg/mL (range, 3.72-10.86 microg/mL), respectively. In all patients with PHPT, the mean leptin levels (34.28 +/- 20.4 ng/mL) were significantly higher than those of HS (P < .01) and, in particular, in PHPT patients with metabolic syndrome (52.63 +/- 31.2 ng/mL) and positively correlated with body mass index, waist circumference, and cholesterol. The mean adiponectin level was significantly lower (4.34 +/- 3.5 mug/mL) only in PHPT patients with metabolic syndrome (P < .005) and negatively correlated with waist circumference and fasting glucose. We concluded that increased serum level of leptin and decreased serum level of adiponectin coexist in patients with PHPT and may represent a pathogenetic factor for cardiovascular disease in this condition.

  20. Reduced immunostaining for the extracellular Ca{sup 2+} - sensing receptor in primary and uremic secondary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Kifor, O.; Moore, F.D. Jr.; Wang, P. [Brigham and Women`s Hospital, Boston, MA (United States)] [and others

    1996-04-01

    Most parathyroid adenomas and some pathological parathyroid glands from patients with primary parathyroid hyperplasia or severe uremic secondary/tertiary hyperparathyroidism show an elevated set-point. In the present study, we investigated whether expression of the Ca{sup 2+}{sub o}-sensing receptor protein recently cloned from bovine parathyroid, a key component in Ca{sup 2+}{sub o}-regulated PTH release, is altered in primary and uremic hyperparathyroidism. Using immunohistochemistry with specific antireceptor antibodies, we compared immunoreactivity of the receptor protein in 14 adenomas, biopsies of 24 normal glands from this same group of patients, and 8 hyperplastic parathyroid glands from 2 individuals with uremic hyparathyroidism. The results show a substantial reduction in the intensity of immunostaining for the receptor protein that averaged nearly 60% for both adenomas and hyperplastic glands, as quantitated by image analysis. There was considerable variation in staining intensity among different pathological parathyroid glands, even in those from the same patient with secondary hyperparathyroidism. In addition, both adenomas and hyperplastic glands had, in some cases, isolated chief cells and groups of cells, sometimes around the periphery of an abnormal gland, with receptor staining equivalent to that of normal parathyroid cells, whereas the bulk of the cells in the same gland showed a marked decrease in staining. Thus, there is a variable, but substantial, reduction in the immunoreactivity of the Ca{sup 2+}{sub o}-sensing receptor protein in both parathyroid adenomas and uremic hyperparathyroidism, as assessed by immunohistochemistry, that probably results from reduced expression of the receptor protein and may contribute to the increase in the set-point often observed in these patients. 49 refs., 8 figs., 1 tab.

  1. A novel SLC12A1 gene mutation associated with hyperparathyroidism, hypercalcemia, nephrogenic diabetes insipidus, and nephrocalcinosis in four patients.

    Science.gov (United States)

    Wongsaengsak, Sariya; Vidmar, Alaina P; Addala, Ananta; Kamil, Elaine S; Sequeira, Paola; Fass, Benjamin; Pitukcheewanont, Pisit

    2017-04-01

    Solute Carrier Family 12 member 1 (SLC12A1) gene encodes the sodium-potassium-chloride co-transporter (NKCC2) at the apical membrane of the thick ascending loop of Henle (TAL). Bartter's syndrome (BS) type I is a rare, autosomal recessive, renal tubular disorder associated with mutation of the SLC12A1 gene. Presenting features include: hypokalemic metabolic alkalosis, hypercalciuria and nephrocalcinosis. The many allelic variants reported present with a spectrum of phenotypes, biochemical abnormalities and clinical severities. However, to date, only two reports have described hyperparathyroidism and hypercalcemia in patients with SLC12A1 gene mutations. We describe 4 patients with 4 novel mutation variants in the SLC12A1 gene (c.735C>G, c.1137del, c.2498-2499del, and c.1833delT) presenting with variable degrees of hyperparathyroidism, hypercalcemia, hypokalemic metabolic alkalosis, nephrocalcinosis, and nephrogenic diabetes insipidus. The link between calcium and parathyroid hormone abnormalities in patients with SLC12A1 mutations is unclear; the cases described suggest an association between primary hyperparathyroidism and loss of function mutation of SLC12A1, which may result in an aberrant threshold of the calcium sensing receptor at the level of the kidney.

  2. Subcutaneous injection is a simple and reproducible option to restore parathyroid function after total parathyroidectomy in patients with secondary hyperparathyroidism.

    Science.gov (United States)

    Ng, Jeremy C F; Wang, Weining; Chua, Min-Jia; Tan, Mui-Suan; Tan, Ngian Chye; Soo, Khee-Chee; Tan, Hiang Khoon; Iyer, N Gopalakrishna

    2014-04-01

    Secondary hyperparathyroidism is a common clinical problem seen in patients with end-stage renal disease (ESRD) undergoing hemodialysis. In patients with severe persistent hyperparathyroidism, parathyroidectomies are often required. We sought to evaluate the feasibility and efficacy of total parathyroidectomy followed by subcutaneous injection of parathyroid autograft compared with surgical implantation. We conducted a retrospective study of 132 patients with confirmed diagnoses of ESRD treated with hemodialysis or peritoneal dialysis, with secondary hyperparathyroidism who had undergone total parathyroidectomies. Clinical and biochemical characteristics, including preoperative and postoperative intact parathyroid hormone levels were recorded and compared between patients who had undergone subcutaneous injection or surgical implantation of autograft. From February 2005 to February 2012, 132 patients who had undergone total parathyroidectomies were included in our study. To compare the techniques of subcutaneous injection and surgical implantation, pre- and postoperative biochemistry was recorded and analyzed. Preoperative biochemistry was comparable in both groups. However, autograft recovery was significantly faster in the group with subcutaneous injection compared with surgical implantation (P = .03). Median time to parathyroid recovery was 2 months for injection compared with 9 months for implantation. There was no remarkable difference in the recurrence rates between the 2 groups. Subcutaneous injection of parathyroid tissue is a feasible and simple alternative to the more commonly used method of surgical implantation. Copyright © 2014 Mosby, Inc. All rights reserved.

  3. 新生儿胆红素脑病33例临床分析%A retrospective study of neonatal severe bilirubin encephalopathy in 33 newborns

    Institute of Scientific and Technical Information of China (English)

    徐瑞峰; 吴珠明; 高红霞; 易彬

    2011-01-01

    目的 探讨新生儿胆红素脑病的病因及临床特点.方法 选择2006年1月至2010年1月我院新生儿科收治的重症新生儿高胆红素血症患儿,分为胆红素脑病组(脑病组)与非胆红素脑病组(非脑病组),比较两组患儿病因、临床特征、胆红素水平、胆红素/白蛋白比值(B/A)以及治疗转归.结果 脑病组黄疸病因以溶血性因素占首位(48.5%),其次是感染因素(24.2%);非脑病组黄疸病因主要为溶血(69.7%).脑病组总胆红素、B/A比值、入院日龄和黄疸持续时间均大于非脑病组[( 555.2±113.9) μmol/L比(431.3±62.3)μmol/L,(0.87±0.17)比(0.67±0.11),(129.5±60.7)h比(53.0±22.6)h,(81.6±39.6)h比(34.2±15.8)h,P均<0.001].胆红素脑病警告期与痉挛期患儿入院日龄、血清胆红素及B/A比值差异无统计学意义(P>0.05).85%的胆红素脑病患儿预后不良.结论 溶血与感染是新生儿胆红素脑病的主要原因,总胆红素浓度过高和干预延迟是引起胆红素脑病的高危因素.%Objective To investigate the etiology and clinical features of neonatal bilirubin encephalopathy. Methods To select severe hyperbilirubinemia infant admitted in Gansu Provincial Maternity and Children's Hospital from January 2006 to January 2010, assigned into encephalopathy group and non-encephalopathy group, comparing their clinical characteristics, bilirubin level, bilirubin/ albumin ratio (B/A) and outcome. Results The main etiology of neonatal bilirubin encephalopathy is hemolytic (48. 5% ) , followed by infection (24. 2% ) ; but the etiology of non-encephalopathy is mainly for hemolysis, accounted for 69. 7%. Total bilirubin, B/A ratio, admission day and jaundice duration of Encephalopathy group are larger than them in non-encephalopathy group, the difference was statistically significant, (555. 2 ±113. 9) μmol/Lvs. (431. 3 ±62. 3) |xmol/L, (0.87 ±0.17) us. (0.67 ±0. 11) , (129.5 ±60.7) h ts. (53.0 ±22.6) h and (81.6 ±39.6) h

  4. Intravenous immunoglobulin therapy in neonatal severe pneumonia%新生儿重症肺炎静脉滴注丙种球蛋白疗效观察

    Institute of Scientific and Technical Information of China (English)

    赵大兴

    2015-01-01

    目的:探讨丙种蛋白静脉注射(IVIG)治疗新生儿重症肺炎的临床疗效。方法将95例新生儿重症肺炎患儿随机分为 IVIG 治疗组和常规治疗组,常规组45例给予抗生素、给氧、血管活性药物,保暖及支持疗法,部分患儿输过1~2次血浆;IVIG 组50例给予常规治疗+ IVIG。结果使用 IVIG 后血清 IgG 水平提高59.46%,而IgA、IgM 无明显改变,补体 C3水平明显降低,T4+细胞和 T4/ T8比例降低,但差异未见统计学意义(P >0.05)。结论 IVIG 在改善症状、消除肺部体征、缩短住院时间和提高治愈率等方面均优于常规治疗。%Objective To investigate the clinical effect of intravenous injection of gamma globu-lin(IVIG)on infants with severe pneumonia. Methods Ninety-five neonatal severe pneumonia patients were randomly divided into IVIG treatment group and routine treatment group,the 45 patients in routine treatment group were given antibiotics,oxygen,vasoactive drugs,warmth and support therapy,some pa-tients had 1 to 2 times infusion of plasma;the 50 patients in IVIG group were given routine treatment +IVIG. Results The level of serum IgG improved by 59. 46% after using IVIG,and IgA,IgM had no obvi-ous change,complement C3 levels decreased significantly,T4 + cells and the T4 / T8 ratio decreased,but there was no significant difference. Conclusions IVIG is superior to routine treatment in improving symp-toms,eliminate the physical signs of the lungs,shorten hospitalization time and improve the cure rate.

  5. Neonatal alloimmune thrombocytopenia

    Directory of Open Access Journals (Sweden)

    Mella MT

    2015-06-01

    Full Text Available Maria Teresa Mella, Keith A Eddleman Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Mount Sinai School of Medicine, New York, NY, USA Abstract: Neonatal alloimmune thrombocytopenia occurs in one in 1,000–1,500 live births and is the most common cause of severe thrombocytopenia and intracranial hemorrhage in term infants. It is the equivalent of red blood cell alloimmunization and is due to transplacental passage of maternal antibodies against paternally derived fetal platelet antigens. A diagnosis of neonatal alloimmune thrombocytopenia should be considered for any neonate with unexplained thrombocytopenia. Once the diagnosis is made, it is known that all subsequent pregnancies are at risk for severe disease. In order to prevent the devastating and potentially life-threatening manifestations of the disease, the goal is to initiate treatment early with serial percutaneous umbilical blood sampling, intravenous immunoglobulin administration, prednisone, and/or fetal platelet transfusions. Timing of delivery is variable with delivery for severe disease recommended at an earlier gestational age. Vaginal delivery can be considered if the fetal platelet count is greater than 50,000–100,000 µL. Thrombocytopenia due to neonatal alloimmune thrombocytopenia usually resolves spontaneously within 1–2 weeks after delivery, but a platelet transfusion may be necessary to prevent a serious hemorrhagic event. In all cases, a multidisciplinary approach to care should be undertaken with delivery at a tertiary care center. Keywords: neonatal thrombocytopenia, fetal therapy, intracranial hemorrhage, intravenous immunoglobulin, alloimmunization

  6. High-intensity focused ultrasound to treat primary hyperparathyroidism: a feasibility study in four patients

    DEFF Research Database (Denmark)

    Kovatcheva, Roussanka D; Vlahov, Jordan D; Shinkov, Alexander D;

    2010-01-01

    Many patients with primary hyperparathyroidism either decline or are not candidates for surgical parathyroidectomy. There are drawbacks to medical therapy as well as percutaneous ethanol injection as alternative therapies for primary hyperparathyroidism. Therefore, in this pilot study, our aim...... was to test the feasibility, safety, and efficacy of a newly developed noninvasive high-intensity focused ultrasound (HIFU) technique for the nonsurgical management of primary hyperparathyroidism....

  7. Simultaneous occurrence of fetal and neonatal alloimmune thrombocytopenia and neonatal neutropenia due to maternal neutrophilic autoantibodies

    DEFF Research Database (Denmark)

    Taaning, Ellen; Jensen, Lise; Varming, Kim

    2012-01-01

    Foetal and neonatal alloimmune thrombocytopenia (FNAIT) and neonatal neutropenia caused by maternal autoantibodies against neutrophils are rare disorders. We describe a newborn with severe thrombocytopenia and intracerebral bleeding caused by maternal anti-HPA-3a alloantibodies and mild neutropenia...

  8. Neonatal and infantile acne vulgaris: an update.

    Science.gov (United States)

    Serna-Tamayo, Cristian; Janniger, Camila K; Micali, Giuseppe; Schwartz, Robert A

    2014-07-01

    Acne may present in neonates, infants, and small children. Neonatal and infantile acne vulgaris are not considered to be rare. The presentation of acne in this patient population sometimes represents virilization and may portend later development of severe adolescent acne. Neonatal and infantile acne vulgaris must be distinguished from other cutaneous disorders seen in newborns and infants. Infantile acne tends to be more pleomorphic and inflammatory, thus requiring more vigorous therapy than neonatal acne.

  9. Tirosinemia neonatal Neonatal tyrosinemia

    Directory of Open Access Journals (Sweden)

    Rafael J. Manotas Cabarcas

    1995-04-01

    Full Text Available Mediante la técnica de Udenfriend y Cooper, se midieron los niveles de tirosina en la sangre del cordón de 26 prematuros y 31 niños de término, con el fin de comparar las concentraciones según la edad gestacional y detectar la presencia de la tirosinemia neonatal. Se encontró un caso de esta entidad en un niño de 31 semanas de edad gestacional, lo cual correspondió al 3.8% de los prematuros y al 1.8% del grupo total. La concentración de tirosina en el paciente fue de 53 JJ.M. El promedio de las concentraciones en los prematuros menores de 32 semanas fue de 16.8 :t 6.3 JJ.M; el de los niños entre 33 y 36 semanas fue de 19.3 :t 7.6 JJ.M y el de los niños de término, de 17.2 :t 9.4 JJ.M. Las pruebas estadísticas no mostraron tendencias ni diferencias significativas entre estas concentraciones. El promedio ponderado para el grupo total fue 17.7 :t 7.3 JJ.M. Se recomienda establecer programas de tamizaje para detectar este problema porque puede presentar repercusiones neurológicas posteriores.

    By means of the Udenfriend-Cooper technique, levels of tyrosine were measured in the cord blood of 26 preterm and 31 term Infants; the objective was to compare tyrosine concentrations according to gestational age and to detect the presence of neonatal tyrosinemia. A case of this disease was found In an Infant with 31 weeks of gestational age; this case represented 3.8% of preterm Infants and 1.8% of the total group. Average tyrosine concentration according to age was as follows: 16.8: ± 6.3  µM in Infants under 32 weeks of gestational age; 19.3: ±: 7.6 µM In those between 33 and 36 weeks and 17.2 : ±: 9.4 µM In the term Infants

  10. Pseudothrombocytopenia in a preterm neonate.

    Science.gov (United States)

    Christensen, Robert D; Sola, Martha C; Rimsza, Lisa M; McMahan, Michael J; Calhoun, Darlene A

    2004-07-01

    Severe and prolonged thrombocytopenia is not uncommon among ill preterm infants. Pseudothrombocytopenia, which has the appearance of severe and prolonged thrombocytopenia, has not been described in this population. We observed a preterm neonate who had EDTA-independent pseudothrombocytopenia and conclude that this condition should be considered when severe and prolonged thrombocytopenia occurs in a neonate in the absence of clinical signs of platelet-type hemorrhage.

  11. Neonatal trauma

    Energy Technology Data Exchange (ETDEWEB)

    Sorantin, Erich [Division of Pediatric Radiology, Department of Radiology, Medical University Graz, Auenbruggerplatz 9, A8036 Graz (Austria)]. E-mail: erich.sorantin@meduni-graz.at; Brader, Peter [Division of Pediatric Radiology, Department of Radiology, Medical University Graz, Auenbruggerplatz 9, A8036 Graz (Austria); Thimary, Felix [Division of Pediatric Radiology, Department of Radiology, Medical University Graz, Auenbruggerplatz 9, A8036 Graz (Austria)

    2006-11-15

    A variety of traumatic lesions can occur during the neonatal period. Some of those lesions are clearly birth injuries due to delivery and others are caused by necessary procedures during intensive care in critically ill neonates. As usual patient history must be known and knowledge about the typical complications is necessary in order to select the appropriate imaging modality and thus enabling correct interpretation of those investigations by the radiologist. The purpose of this article is to present typical neonatal injuries, describe the underlying pathomechanisms and aetiology as well as the imaging findings.

  12. Effect of gastric bypass on vitamin D and secondary hyperparathyroidism.

    Science.gov (United States)

    Signori, Carina; Zalesin, Kerstyn C; Franklin, Barry; Miller, Wendy L; McCullough, Peter A

    2010-07-01

    Obesity as well as bariatric surgery may increase the risk for vitamin D deficiency. We retrospectively compared vitamin D levels in obese patients (n = 123) prior to bariatric surgery and 1 year postoperatively. We also evaluated parathyroid hormone levels (PTH) 1 year after surgery. A higher percentage of patients had baseline vitamin D deficiency (86%), defined as 25-hydroxy vitamin D vitamin D deficiency at baseline (r = -0.3, p = 0.06) and at the postoperative follow-up (r = -0.2, p = 0.013). One third of the postoperative population had secondary hyperparathyroidism, defined by a serum PTH level >62 pg/mL; however, postoperative PTH and vitamin D levels were unrelated (r = -0.001, p = 0.994). Pre- and postoperative vitamin D levels were inversely correlated with BMI. Secondary hyperparathyroidism was observed in 33% of patients postoperatively; however, this did not correlate with vitamin D.

  13. [Brown bone tumor as the first manifestation of primary hyperparathyroidism].

    Science.gov (United States)

    Marcos García, M; Pino Rivero, V; Keituqwa Yáñez, T; Alcaraz Fuentes, M; Trinidad Ruiz, G; Blasco Huelva, A

    2003-01-01

    We report a clinical case of a 26 years old female who had a 2 years evolution chin tumour with hypercalcemia (11.8 mg/dl) and PTH (paratohormone) of 761 pg/ml. She underwent a CT scan and MRI of the mandible, as well as a biopsy followed by excision of the tumour by the maxilofacial surgeons. Our ENT Department asked for a Scintigraphy (Tc99s-mibi) and thoracic-cervical CT, which showed a lesion that turned out to be an adenoma of the lower right parathyroid gland after surgery and pathological examination. The patient suffered a Primary hyperparathyroidism that was the main stimulus for the Brown Tumour made up by macrophagos and multinuclear giant cells, being this the first manifestation of the metabolic disorder. This form of hyperparathyroidism is very rare in the clinic. We do a literature review to establish the differential diagnosis for such pathology.

  14. Bilateral genu valgum: an unusual presentation of juvenile primary hyperparathyroidism.

    Science.gov (United States)

    Sharma, Shruti; Kumar, Sunil

    2016-07-01

    Primary hyperparathyroidism is a generalized disorder of bone and mineral metabolism caused by autonomous secretion of parathyroid hormone. It is primarily seen in adults with typical age of presentation between third and fifth decades of life. Juvenile hyperparathyroidism is a rare disorder. The common presentations in order of incidence are fatigue and lethargy, headache, nephrolithiasis, nausea, abdominal pain, vomiting and polydipsia. Though skeletal symptoms include bone pains and fractures, but the presence of limb deformity is atypical. We report a case of young girl who presented with isolated progressive genu valgum of both lower limbs and pigeon-shaped chest deformity. She was found to have hypercalcemia and hypophosphatemia with raised parathyroid hormone levels. The neck imaging showed a single adenoma in the left inferior parathyroid gland. The surgical removal of parathyroid adenoma was performed.

  15. Brown tumor of the maxilla in patient with secondary hyperparathyroidism

    OpenAIRE

    2004-01-01

    Brown tumor or parathyroid osteopathy is a kind of bony lesion caused by hyperparathyroidism. It appears as an expansive osteolytic lesion mostly in mandible, ribs, pelvis and femur, but rarely in the upper jaw. Bone resorption is the result of osteoclastic activity due to an increased activity of parathyroid hormone. A 25-years-old male patient was operated on due to clinicaly and radiographicaly obvious maxillary tumor and increased values of parathyroid hormon (PTH - 1 050 ng/l). The level...

  16. Management of hyperparathyroidism (PHP) in MEN2 syndromes in Europe.

    Science.gov (United States)

    Alevizaki, Maria

    2013-03-14

    Hyperparathyroidism occurs in 20-30% of MEN2A syndrome patients. It is usually associated with mild disease and is frequently asymptomatic, especially in younger age. There is genotype/phenotype association and PHP is usually associated with codon 634 mutations; however association with more "rare" mutations has also been reported. The pathology of the parathyroid glands includes hyperplasia, adenoma or a combination of the two. The optimal surgical management of this entity has not been defined yet.

  17. Neonatal Jaundice

    DEFF Research Database (Denmark)

    Maimburg, Rikke Damkjær; Væth, Michael; Schendel, Diana

    2008-01-01

    .7]). No associations were found between infantile autism and low Apgar scores, acidosis or hypoglycaemia. Our findings suggest that hyperbilirubinaemia and neurological abnormalities in the neonatal period are important factors to consider when studying causes of infantile autism....

  18. Neonatal Death

    Science.gov (United States)

    ... a premature baby include pneumonia (a lung infection), sepsis (a blood infection) and meningitis (an infection in the fluid around the brain and spinal cord). What birth defects most often cause neonatal death? The most common birth defects that cause ...

  19. A Case of Primary Hyperparathyroidism due to Intrathyroidal Parathyroid Cyst

    Directory of Open Access Journals (Sweden)

    Yavuz Yalcin

    2014-01-01

    Full Text Available Parathyroid cysts constitute 0.08–3.41% of all parathyroid masses. Intrathyroidal parathyroid cysts, however, are rare conditions with only a few cases being reported. Most of the parathyroid cysts are found to be nonfunctional and functional cysts are generally thought to be due to cystic degeneration of parathyroid adenomas. A cystic, smooth contoured lesion of 24 × 19 × 16 mm was observed in left thyroid lobe of a 76-year-old woman during ultrasonography which was performed as routine workup for primary hyperparathyroidism. It was defined as a cystic thyroid nodule at first. Tc99m sestamibi scintigraphy was performed to see any parathyroid lesions, but no radioactive uptake was observed. Intact parathormone (iPTH level was found to be >600 pg/mL in cyst aspiration fluid. Left lobectomy was performed, with a diagnosis of primary hyperparathyroidism due to functional parathyroid cyst. Serum iPTH level was decreased >50% postoperatively and histopathological evaluation was consistent with an encapsulated parathyroid adenoma with a cystic center. Parathyroid cysts are among rare causes of primary hyperparathyroidism. Diagnosis is made by markedly increased iPTH level in cyst fluid and observation of parathyroid epithelium lining the cyst wall.

  20. Management of Primary Hyperparathyroidism: Can We Do Better?

    Science.gov (United States)

    Sharata, Ahmed; Kelly, Tracy L; Rozenfeld, Yelena; Hammill, Chet W; Schuman, Earl; Carlisle, James R; Aliabadi-Wahle, Shaghayegh

    2017-01-01

    The failure to follow national guidelines in management of various diseases has been previously established. We sought to quantify primary care providers' familiarity with primary hyperparathyroidism as it affects adherence to the 2009 National Institute of Health (NIH) consensus recommendations in treatment of primary hyperparathyroidism. A large primary care group was surveyed to determine their familiarity with the 2009 NIH consensus recommendations for management of primary hyperparathyroidism (PHPT). Retrospective review of the group's records (2009-2011) was performed to verify compliance. Survey responders included 109 clinicians, 31 per cent were familiar with all criteria for surgical intervention in asymptomatic patients and 34 per cent correctly identified appropriate surveillance testing for patients undergoing observation. Chart review identified 124 patients with PHPT. Of the patients who met NIH criteria, 34 per cent had a parathyroidectomy. Younger age, higher intact parathyroid hormone, hypercalciuria, and history of nephrolithiasis were associated with surgery in multivariable analysis. Of the observed patients, 16 per cent had appropriate surveillance studies. In conclusion, this study confirms suboptimal adherence with consensus recommendations in management of PHPT. A minority of clinicians demonstrated solid familiarity with management strategies, paralleling their treatment approach. Educational efforts may improve adherence with upcoming national recommendations.

  1. 825例重度子痫前期患者的分娩时机与围产儿结局分析%Analysis the ultimate time of severe preeclampsia 566 cases and the neonatal outcomes in Xinjiang province

    Institute of Scientific and Technical Information of China (English)

    王志梅; 韩莉莉

    2012-01-01

    Objective: To investigate the severe preeclampsia and neonatal outcome. Methods; 566 patients with severs pre-eclampsia were generally treated according to the condition of patients, looking forward to treatment and termination of pregnancy. A-nalysis of different treatment regimens neonatal outcome. Results. The gestational age > 32 weeks of the neonatal and perinatal mortality of children was obvious lower than 32 weeks before the termination of pregnancy (P < 0.01) Conclusions: Nearly 34 weeks gestation and early onset sever preeclampsia. Pregnancy can be terminated by promoting the maturity of fetal lung 32weeks of pregnancy before the early onset severe preeclampsia, maternal complications was increasing with prolonged gestational ages under strict monitor.%目的 探讨重度子痫前期孕妇的分娩时机及新生儿的结局.方法 对825例重度子痫前期的患者,根据病情进一步治疗,期待治疗和终止妊娠,分析不同治疗方法的预后.结果 孕周> 32周的新生儿和围产儿病死率低于32周前终止妊娠者,(P<0.01)结论 孕周已近34周的重度子痫前期患者,经促胎肺治疗后即可终止妊娠.而孕32周之前的重度子痫前期患者,在严密监测下尽量延长孕周,以增加胎儿的成熟度并不增加母亲并发症.

  2. Hiperbilirrubinemia neonatal agravada Aggravated neonatal hyperbilirubinemia

    Directory of Open Access Journals (Sweden)

    Ana Campo González

    2010-09-01

    Full Text Available INTRODUCCIÓN. La mayoría de las veces la ictericia en el recién nacido es un hecho fisiológico, causado por una hiperbilirrubinemia de predominio indirecto, secundario a inmadurez hepática e hiperproducción de bilirrubina. El objetivo de este estudio fue determinar el comportamiento de la hiperbilirrubinemia neonatal en el Hospital Docente Ginecoobstétrico de Guanabacoa en los años 2007 a 2009. MÉTODOS. Se realizó un estudio descriptivo y retrospectivo de 173 recién nacidos que ingresaron al Departamento de Neonatología con diagnóstico de hiperbilirrubinemia agravada. RESULTADOS. La incidencia de hiperbilirrubinemia neonatal agravada fue del 3,67 % y predominó en hermanos con antecedentes de ictericia (56,65 %. El tiempo de aparición fue de 48 a 72 h (76,87 % y entre los factores agravantes se hallaron el nacimiento pretérmino y el bajo peso al nacer. La mayoría de los pacientes fueron tratados con luminoterapia (90,17 %. CONCLUSIÓN. La hiperbilirrubinemia neonatal agravada constituye un problema de salud. Los factores agravantes son la prematuridad y el bajo peso al nacer. La luminoterapia es una medida terapéutica eficaz para su tratamiento.INTRODUCTION. Most of times jaundice in newborn is a physiological fact due to hyperbilirubinemia of indirect predominance, secondary to liver immaturity and to bilirubin hyperproduction. The aim of present of present study was to determine the behavior of neonatal hyperbilirubinemia in the Gynecology and Obstetrics Teaching Hospital of Guanabacoa municipality from 2007 to 2009. METHODS. A retrospective and descriptive study was conducted in 173 newborn patients admitted in the Neonatology Department diagnosed with severe hyperbilirubinemia. RESULTS. The incidence of severe neonatal hyperbilirubinemia was of 3,67% with predominance in brothers with a history of jaundice (56,65%. The time of appearance was of 48 to 72 hrs (76,87% and among the aggravating factors were the preterm birth and

  3. 输液泵控制换血术治疗新生儿重症黄疸的护理研究%Nursing of Infusion Pump Control Transfusion in Treating Severe Neonatal Jaundice

    Institute of Scientific and Technical Information of China (English)

    耿洪维

    2016-01-01

    目的:探讨输液泵控制换血术治疗新生儿重症黄疸的护理效果。方法将68例新生儿重症黄疸患儿随机分为对照组和研究组,各34例,两组均接受输液泵控制换血术治疗,对照组应用常规护理,研究组应用综合护理,比较两组护理效果。结果两组患者临床治疗护理效果比较,研究组高于对照组,差异具有统计学意义(P<0.05)。结论应用输液泵控制换血术治疗新生儿重症黄疸能够取得显著的效果,配合综合护理有助于提高治疗效果。%Objective To investigate the nursing effect of infusion pump controlled exchange transfusion for treatment of neonatal severe jaundice. Methods 68 cases of neonatal severe jaundice patients were randomly assigned to study group and control group, 34 cases in each group, two groups underwent infusion pump control exchange transfusion for treatment, the control group used routine nursing, the research group was treated with comprehensive nursing, compared two groups of nursing effect. Results Two groups of patients with clinical treatment effect comparison, the study group was significantly higher than the control group, the difference was statistically significant (P<0.05).Conclusion By infusion pump control exchange transfusion in treating severe neonatal jaundice can achieve significant results, with comprehensive nursing is helpful to improve the therapeutic effect.

  4. Novel deletion and a new missense mutation (Glu 217 Lys) at the catalytic site in two adenosine deaminase alleles of a patient with neonatal onset adenosine deaminase severe combined immunodeficiency

    Energy Technology Data Exchange (ETDEWEB)

    Hirschhorn, R.; Nicknam, M.N.; Eng, F.; Yang, D.R.; Borkowsky, W. (New York Univ. Medical School of Medicine, NY (United States))

    1992-11-01

    Mutations at the adenosine deaminase (ADA) locus result in a spectrum of disorders, encompassing a fulminant neonatal onset severe combined immunodeficiency (SCID) and childhood onset immunodeficiency, as well as apparently normal immune function. The extent of accumulation of the toxic metabolite, deoxyATP, correlates directly with severity of disease. The authors have now determined the mutations on both alleles of a child with fulminant, neonatal onset ADA SCID and accumulation of extremely high concentrations of deoxyATP. The genotype was consistent with the severely affected phenotype. One allele carried a large deletion that arose by non-homologous recombination and included the first five exons and promoter region. The second allele carried a missense mutation (G[sup 649]A) resulting in replacement of Glu[sup 217], an amino acid involved in the catalytic site, by Lys and predicting a major alteration in charge. Expression of the mutant cDNA on Cos cells confirmed that the mutation abolished enzyme activity. The authors have previously reported that a missense mutation at the preceding codon is similarly associated with neonatal onset ADA SCID and accumulation of extremely high deoxyATP. These findings suggest that genotype-phenotype correlations may be apparent for ADA SCID, despite the role that random variation in exposure to environmental pathogens may play in the initial phenotype. Such genotype-phenotype correlations may be important to consider in evaluating results of ongoing trials of [open quotes]gene[close quotes] and enzyme replacement therapy. 50 refs., 5 figs., 2 tabs.

  5. Primary hyperparathyroidism by parathyroid gland adenoma (Report of 2 cases with review of the literature)

    Energy Technology Data Exchange (ETDEWEB)

    Cha, Seong Sook; Han, Sang Suk [Inje Medical College, Busan (Korea, Republic of); Chae, Yoo Soon [Maryknoll Medical Center, Busan (Korea, Republic of)

    1985-02-15

    The primary hyperparathyroidism is a complex endocrine disease caused by neoplasm or diffuse hyperplasia of parathyroid gland in which excessive parathyroid hormone is secreted. This results in chemical abnormalities of serum, and exerts major influences on the bone, kidney and gastrointestinal tract. The authors report 2 cases of primary hyperparathyroidism with review of the literature.

  6. Bilateral simultaneous quadriceps tendon rupture in a patient with secondary hyperparathyroidism: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yeon Soo; Son, Sang Beom; Han, Chang Whan; Kang, Si Won [Taejon St. Mary' s Hospital, The Catholic Univ. of Korea, Taejon (Korea, Republic of)

    2001-11-01

    Simultaneous bilateral rupture of the quadriceps tendon without a significant history of trauma may occur in association with chronic metabolic disorders such as chronic renal failure and secondary hyperparathyroidism, though has rarely been reported. We describe a case of spontaneous bilateral quadriceps tendon rupture in a 36-year-old female patient with secondary hyperparathyroidism.

  7. Aortopulmonary window parathyroid gland causing primary hyperparathyroidism in men type 1 syndrome.

    Science.gov (United States)

    Tonelli, Francesco; Biagini, Carlo; Giudici, Francesco; Cioppi, Federica; Brandi, Maria Luisa

    2016-01-01

    Primary hyperparathyroidism (HPT) is the most common endocrinopathy in Multiple Endocrine Neoplasia type 1 (MEN1) syndrome. Supernumerary and/or ectopic parathyroid glands, potentially causes of persistent or recurrent HPT after surgery, have been previously described. However, this is the first ever described case of ectopic parathyroid gland localized in the aortopulmunary window causing HPT in MEN1. After a consistent concordant pre-operative imaging assessment the patient, a 16 years old male affected by a severe hypercalcemia, underwent surgery. The parathyroid was found very deeply near the tracheal bifurcation, hidden by the aortic arch itself and for this reason not visible at the beginning of the dissection but only after being identified by palpation for its typical consistence. The intraoperative PTH decreased at normal level 10 min after removal of the ectopic gland. The patient remained with normal value of calcemia and PTH during the 10 months of follow-up.

  8. Primary Hyperparathyroidism in Pregnancy: A Two-Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    A. D. Herrera-Martínez

    2015-01-01

    Full Text Available Primary hyperparathyroidism (PHPT in pregnant women is an uncommon disease. It could be easily misdiagnosed because of physiologic changes during pregnancy; in some cases, patients could remain asymptomatic maintaining elevated calcium serum levels, and this situation represents a threat to the health of both mother and fetus. We present two cases of PHPT during pregnancy and their evolution after surgical treatment in the second trimester; there were no observed complications during pregnancy or delivery in our patients. Early diagnosis and medical/surgical treatment in PHPT are necessary for avoiding maternal and fetal complications which could not be predicted based on duration or severity of hypercalcemia. An appropriate management of PHPT during pregnancy is necessary for preserving the health of both the woman and the fetus.

  9. Autotransplant tissue selection criteria with or without stereomicroscopy in parathyroidectomy for treatment of renal hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Monique Nakayama Ohe

    2014-07-01

    Full Text Available INTRODUCTION: Several methods have been proposed to improve operative success in renal hyperparathyroidism. OBJECTIVE: To evaluate stereomicroscopy in parathyroid tissue selection for total parathyroidectomy with autotransplantation in secondary (SHPT/tertiary (THPT hyperparathyroidism. METHODS: 118 renal patients underwent surgery from April of 2000 to October 2009. They were divided into two groups: G1, 66 patients operated from April of 2000 to May of 2005, with tissue selection based on macroscopic observation; G2, 52 patients operated from March of 2008 to October 2009 with stereomicroscopy for tissue selection searching for the presence of adipose cells. All surgeries were performed by the same surgeon. Patients presented SHPT (dialysis treatment or THPT (renal-grafted. Follow-up was 12-36 months. Intra-operative parathyroid hormone (PTH was measured in 100/118 (84.7% patients. RESULTS: Data are presented as means. G1 included 66 patients (38 SHPT, 24 females/14 males; 40.0 years of age; 28 THPT, 14 females/14 males; 44 years of age. G2 included 52 patients (29 SHPT, 11 females/18 males; 50.7 years of age; 23 THPT, 13 females/10 males, 44.4 years of age. SHPT patients from G2 presented preoperative serum calcium higher than those of SHPT patients in G1 (p < 0.05, suggesting a more severe disease. Definitive hypoparathyroidism was found in seven of 118 patients (5.9%. Graft-dependent recurrence occurred in four patients, two in each group. All occurred in dialysis patients. CONCLUSION: Stereomicroscopy in SHPT/THPT surgical treatment may be a useful tool to standardize parathyroid tissue selection.

  10. Hyperparathyroid crisis due to asymmetric parathyroid hyperplasia with a massive ectopic parathyroid gland.

    Science.gov (United States)

    Gratian, Lauren F; Hyland, Kristen A; Scheri, Randall P

    2014-10-01

    To report a rare case of primary hyperparathyroidism presenting with hyperparathyroid crisis due to parathyroid hyperplasia with ectopic glands. We present the initial clinical manifestations, laboratory results, radiologic and surgical findings, and management in a patient who had hyperparathyroid crisis. The pertinent literature and management options are also reviewed. A 60-year-old female presented with hyperparathyroid crisis requiring preoperative stabilization with rehydration, diuresis, bisphosphonate therapy, and ultimately hemodialysis. Parathyroidectomy revealed asymmetric 4-gland hyperplasia, with a massive ectopic parathyroid gland in the tracheoesophageal groove extending into the mediastinum. Her postoperative course was complicated by hungry bone syndrome and hypocalcemia. This case illustrates the rare occurrence of hyperparathyroid crises due to asymmetric parathyroid hyperplasia with a massive ectopic parathyroid gland.

  11. Influence of age and gender on presentation of symptomatic primary hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    V N Shah

    2012-01-01

    Full Text Available Background: The geographical difference in presentation of primary hyperparathyroidism (PHPT is known. However, there is sparse literature on the influence of age and gender on presentation of PHPT. Aim: To analyze the effect of age and gender on presentation of symptomatic primary hyperparathyroidism. Setting and Design: This is a retrospective analysis of data from the primary hyperparathyroidism registry of a north Indian tertiary care teaching institute. Materials and Methods: Analysis of 184 histopathologically proven PHPT patients registered between March 1990 and March 2010 from a single centre of north India. PHPT patients were divided into three different age groups i.e. children and adolescents less than 25 years, adults 25-49 years, and ≥ 50 years. Clinical presentations, biochemical parameters and parathyroid weight were compared between different age groups and gender using appropriate statistical methods. Results: Mean age of patients was 38.5±13.8 years with female: male ratio of 7:3. Rickets as presenting manifestations were seen in one child and adolescent each. Prevalence of renal stones (P=0.03 and gall stones (P=0.02 was higher in the adult groups compared to the younger and older. There was no difference in bone pain (P=0.7, fracture (P=0.3, osteitis fibrosa cystica (P=0.2, fatigue (P=0.6 and other symptoms among different age groups. There was no difference in serum calcium, phosphate, parathyroid hormone (PTH and 25 (OH D levels among different age groups, however, as expected alkaline phosphatase was higher in adolescents compared to adults (P=0.03. Bone pain and muscle aches (P<0.001, fracture (P=0.04, osteitis fibrosa cystica (P=0.01, and gall stones (P=0.03 were more common among women while renal stones (P=0.05 and pancreatitis (P=0.02 were common in men. Serum calcium and phosphate levels were similar in either sex but parathyroid hormone (iPTH level was higher among women (P=0.02. Parathyroid adenoma weight was

  12. Neonatal neurosonography

    Energy Technology Data Exchange (ETDEWEB)

    Riccabona, Michael, E-mail: michael.riccabona@klinikum-graz.at

    2014-09-15

    Paediatric and particularly neonatal neurosonography still remains a mainstay of imaging the neonatal brain. It can be performed at the bedside without any need for sedation or specific monitoring. There are a number of neurologic conditions that significantly influence morbidity and mortality in neonates and infants related to the brain and the spinal cord; most of them can be addressed by ultrasonography (US). However, with the introduction of first CT and then MRI, neonatal neurosonography is increasingly considered just a basic first line technique that offers only orienting information and does not deliver much relevant information. This is partially caused by inferior US performance – either by restricted availability of modern equipment or by lack of specialized expertise in performing and reading neurosonographic scans. This essay tries to highlight the value and potential of US in the neonatal brain and briefly touching also on the spinal cord imaging. The common pathologies and their US appearance as well as typical indication and applications of neurosonography are listed. The review aims at encouraging paediatric radiologists to reorient there imaging algorithms and skills towards the potential of modern neurosonography, particularly in the view of efficacy, considering growing economic pressure, and the low invasiveness as well as the good availability of US that can easily be repeated any time at the bedside.

  13. Neonatal pain.

    Science.gov (United States)

    Walker, Suellen M

    2014-01-01

    Effective management of procedural and postoperative pain in neonates is required to minimize acute physiological and behavioral distress and may also improve acute and long-term outcomes. Painful stimuli activate nociceptive pathways, from the periphery to the cortex, in neonates and behavioral responses form the basis for validated pain assessment tools. However, there is an increasing awareness of the need to not only reduce acute behavioral responses to pain in neonates, but also to protect the developing nervous system from persistent sensitization of pain pathways and potential damaging effects of altered neural activity on central nervous system development. Analgesic requirements are influenced by age-related changes in both pharmacokinetic and pharmacodynamic response, and increasing data are available to guide safe and effective dosing with opioids and paracetamol. Regional analgesic techniques provide effective perioperative analgesia, but higher complication rates in neonates emphasize the importance of monitoring and choice of the most appropriate drug and dose. There have been significant improvements in the understanding and management of neonatal pain, but additional research evidence will further reduce the need to extrapolate data from older age groups. Translation into improved clinical care will continue to depend on an integrated approach to implementation that encompasses assessment and titration against individual response, education and training, and audit and feedback.

  14. Ictericia Neonatal

    OpenAIRE

    Blanco de la Fuente, María Isabel

    2014-01-01

    El motivo que ha llevado a la realización de este trabajo fin de grado sobre el tema de la ICTERICIA NEONATAL se debe a la elevada frecuencia de su aparición en la población. Un porcentaje elevado de RN la padecen al nacer siendo, en la mayor parte de los casos, un proceso fisiológico resuelto con facilidad debido a una inmadurez del sistema hepático y a una hiperproducción de bilirrubina. La ictericia neonatal es la pigmentación de color amarillo de la piel y mucosas en ...

  15. Ethical issues in neonatal intensive care

    OpenAIRE

    Marcello M. Orzalesi; Marina Cuttini

    2011-01-01

    Recent progress in neonatal care have significantly improved the prognosis and chances of survival of critically ill or extremely preterm neonates and have modified the limits of viability. However, in some circumstances, when the child's death can only be briefly postponed at the price of severe suffering, or when survival is associated with severe disabilities and an intolerable life for the child and his/her parents, the application of the full armamentarium of modern neonatal intensive ca...

  16. Association of heart rate variability with 5-minute Apgar score in neonates with severe asphyxia%重度窒息新生儿5分钟Apgar评分的心率变异性分析

    Institute of Scientific and Technical Information of China (English)

    王瑾; 李丽; 阚亚楠; 梁宏伟; 陈家菲

    2013-01-01

    目的探讨重度窒息新生儿5 min Apgar评分与心率变异性(HRV)的关系。方法入选103例出生后1 min Apgar评分为0~3分的重度窒息新生儿,根据出生后5 min Apgar评分分组,>7分为A组(n=50),≤7分B组(n=53);同时以40例1、5 min Apgar评分均>7分的足月新生儿作为对照组;三组新生儿均于出生后第3天行24 h动态心电图检查,并分析其HRV变化。结果 B组较对照组及A组HRV时域指标PNN50、rMSSD、SDSD降低,SDNN、SDANN升高,差异均有统计学意义(P0.05)。结论新生儿窒息损伤自主神经功能,5 min Apgar评分联合HRV时域参数可作为重度窒息新生儿自主神经功能损伤及预后的无创判断指标。%Objective To explore the relationship between 5-minute Apgar score and heart rate variability (HRV) in severely asphyxiated neonates. Methods A total of 103 severely asphyxiated neonates with 1-minute Apgar score of 0 to 3 points were selected. They were divided into Group A (>7 points, n=50) and Group B (≤7 points, n=53) based on 5-minute Apgar score. Meanwhile, 40 full-term neonates with 1-and 5-minute Apgar score greater than 7 points were selected as control group. 24-hour dynamic electrocardiogram was performed and HRV was analyzed on the third day after birth in three groups. Results PNN50, rMSSD, SDSD were decreased and SDNN, SDANN were increased in group B as compared with group A and control group (P0.05). Conclusions Neonatal asphyxia can cause damage to autonomic nervous system. 5-minute Apgar score and HRV can be joint-ly used as a non-invasive index in autonomic nervous damage and its prognosis in asphyxiated newborns.

  17. Clinical features and maternal and neonatal outcomes of severe preeclampsia%80例重度子痫前期的临床特点和妊娠结局分析

    Institute of Scientific and Technical Information of China (English)

    梅吉; 于红

    2012-01-01

    Objective: To explore clinical features and maternal and neonatal outcomes of early and late onset severe preeclampsia. Methods: Clinical data of 80 cases of severe preeclampsia in Southeast University Jiangyin Hospital from January 2010 to February 2012, were collected and then analyzed. The patients were divided into two groups, 20 cases of early onset severe preeclampsia group (onset gestational weeks < 34) and 60 cases of later onset severe preeclampsia group (onset gestational weeks 3= 34). Results: The rates of complications of early onset severe preeclampsia group were higher than that of later onset severe preeclampsia group , and the rate of cesarean section in early onset severe preeclampsia group were higher than that of later onset severe preeclampsia group with an obvious differences ( all P < 0. 05 ). Significant difference of two groups were found in the rate of fetal distress, dead fetus in uterus, FGR, neonatal asphyxia and neonatal death(P <0. 01). Conclusions: Clinical symptoms of early onset severe preeclampsia are more serious, and have a higher incidence of abnormal maternal and neonatal outcomes. The suitable duration of expectant management can improve maternal- prenatal prognosis. Cesarean section is the major method of terminating severe preeclampsia.%目的:探讨早发型及晚发型重度子痫前期的临床特点及围产结局.方法:回顾性分析东南大学附属江阴医院产科2010年1月至2012年2月收治的重度子痫前期80例患者的临床资料,将其分为早发型重度子痫前期组(早发型组,妊娠<34周,20例)和晚发型重度子痫前期组(晚发型组,妊娠≥34周,60例).结果:孕产妇并发症早发型组高于晚发型组,终止妊娠的孕周早于晚发型组,终止妊娠的方式剖宫产率高于晚发型组,差异均具有统计学意义(P<0.05);胎儿窘迫、死胎、胎儿生长受限、新生儿窒息、新生儿死亡等发生率早发型组显著高于晚发型组(P<0.01).结论

  18. Neonatal hematology.

    Science.gov (United States)

    Diaz-Miron, Jose; Miller, Jacob; Vogel, Adam M

    2013-11-01

    Neonatal hematology is a complex and dynamic process in the pediatric population. Surgeons frequently encounter hematologic issues regarding hemostasis, inflammation, and wound healing. This publication provides a surgeon-directed review of hematopoiesis in the newborn, as well as an overview of the current understanding of their hemostatic profile under normal and pathologic conditions. © 2013 Published by Elsevier Inc.

  19. Neonatal Kraniefraktur

    DEFF Research Database (Denmark)

    Johannesen, Katrine Marie Harries; Stantchev, Hristo

    2015-01-01

    During the latest decades the incidence of birth traumas has decreased significantly. Even so the traumas still contribute to an increased mortality and morbidity. We present a case of spontaneous neonatal skull fracture following a normal vaginal delivery. Abnormal facial structure was seen...

  20. Association between long-term efficacy of cinacalcet and parathyroid gland volume in haemodialysis patients with secondary hyperparathyroidism.

    Science.gov (United States)

    Tanaka, Motoko; Nakanishi, Shohei; Komaba, Hirotaka; Itoh, Kazuko; Matsushita, Kazutaka; Fukagawa, Masafumi

    2008-08-01

    Purpose. Secondary hyperparathyroidism with nodular hyperplasia is resistant to medical therapies. Cinacalcet is an effective treatment for severe secondary hyperparathyroidism. This multicentre retrospective study was designed to determine the long-term efficacy of cinacalcet in patients with nodular hyperplasia, the advanced type of parathyroid hyperplasia. Subjects and methods. The study subjects were 20 haemodialysis patients with secondary hyperparathyroidism. Patients with ultrasonographically confirmed large parathyroid glands (volume >0.5 cm(3)) were considered to have nodular hyperplasia (n = 8). Cinacalcet was started at the dose of 25 mg/day and titrated up to 100 mg/day to achieve the target intact-parathyroid hormone (iPTH) level of <250 pg/ml. Serum iPTH, corrected calcium, serum phosphorus, calcium × phosphorus product were measured and compared over the 48-week period of treatment with cinacalcet in all 20 patients and over 120 weeks in 6 of the patients (2 with nodular hyperplasia and 4 with non-nodular hyperplasia). We also examined the achievement rate of K/DOQI guideline treatment targets. The dosages of vitamin D preparation, sevelamer hydrochloride and calcium- containing phosphate binder were adjusted for the above target values. Results. iPTH levels were significantly lower at 48 weeks in both groups. However, corrected calcium levels, serum phosphorus levels and calcium phosphorus products were within the target values in the non-nodular hyperplasia group (n = 12), while the target value could not be achieved in the nodular hyperplasia group. In the long-term follow-up group, the levels of iPTH, corrected calcium, serum phosphorus and calcium × phosphorus products were significantly higher in nodular hyperplasia than in non-nodular hyperplasia. Conclusion. Our study suggests that cinacalcet lacks long-term efficacy in nodular hyperplasia, especially for controlling serum calcium and phosphorus levels.

  1. Association between long-term efficacy of cinacalcet and parathyroid gland volume in haemodialysis patients with secondary hyperparathyroidism

    Science.gov (United States)

    Tanaka, Motoko; Nakanishi, Shohei; Komaba, Hirotaka; Itoh, Kazuko; Matsushita, Kazutaka; Fukagawa, Masafumi

    2008-01-01

    Purpose. Secondary hyperparathyroidism with nodular hyperplasia is resistant to medical therapies. Cinacalcet is an effective treatment for severe secondary hyperparathyroidism. This multicentre retrospective study was designed to determine the long-term efficacy of cinacalcet in patients with nodular hyperplasia, the advanced type of parathyroid hyperplasia. Subjects and methods. The study subjects were 20 haemodialysis patients with secondary hyperparathyroidism. Patients with ultrasonographically confirmed large parathyroid glands (volume >0.5 cm3) were considered to have nodular hyperplasia (n = 8). Cinacalcet was started at the dose of 25 mg/day and titrated up to 100 mg/day to achieve the target intact-parathyroid hormone (iPTH) level of <250 pg/ml. Serum iPTH, corrected calcium, serum phosphorus, calcium × phosphorus product were measured and compared over the 48-week period of treatment with cinacalcet in all 20 patients and over 120 weeks in 6 of the patients (2 with nodular hyperplasia and 4 with non-nodular hyperplasia). We also examined the achievement rate of K/DOQI guideline treatment targets. The dosages of vitamin D preparation, sevelamer hydrochloride and calcium- containing phosphate binder were adjusted for the above target values. Results. iPTH levels were significantly lower at 48 weeks in both groups. However, corrected calcium levels, serum phosphorus levels and calcium phosphorus products were within the target values in the non-nodular hyperplasia group (n = 12), while the target value could not be achieved in the nodular hyperplasia group. In the long-term follow-up group, the levels of iPTH, corrected calcium, serum phosphorus and calcium × phosphorus products were significantly higher in nodular hyperplasia than in non-nodular hyperplasia. Conclusion. Our study suggests that cinacalcet lacks long-term efficacy in nodular hyperplasia, especially for controlling serum calcium and phosphorus levels. PMID:25983974

  2. [Hyperparathyroidism disclosed by forward sagging of the head].

    Science.gov (United States)

    Berenbaum, F; Rajzbaum, G; Bonnichon, P; Amor, B

    1993-06-01

    An unusual clinical presentation of hyperparathyroidism is reported. The 73-year-old patient was unable to maintain her head upright after ten minutes of walking or standing. Parathyroid adenoma was diagnosed on the basis of ultrasound and pathological findings and parathyroid hormone assays. Following surgery the forward sagging of the head no longer occurred and serum levels of calcium and phosphorus returned to normal. Pathophysiological hypotheses are discussed. No similar cases with isolated weakness of the cervical and dorsal paravertebral muscles has been reported to date.

  3. 18F-FET-PET in Primary Hyperparathyroidism

    DEFF Research Database (Denmark)

    Krakauer, Martin; Kjær, Andreas; Bennedbæk, Finn Noe

    2016-01-01

    Preoperative localisation of the diseased parathyroid gland(s) in primary hyperparathyroidism (PHP) is a prerequisite for subsequent minimally invasive surgery. Recently, as alternatives to conventional sestamibi parathyroid scintigraphy, the (11)C-based positron emission tomography (PET) tracers......-isotope parathyroid subtraction single photon emission computed tomography had determined the exact location of the parathyroid adenoma. A dynamic FET PET/CT scan was performed with subsequent visual evaluation and calculation of target-to-background (TBR; parathyroid vs. thyroid). The maximum TBR in the two patients...

  4. 重症新生儿肺炎的急救措施及护理措施总结%First Aid Measures and Nursing Measures of Severe Neonatal Pneumonia

    Institute of Scientific and Technical Information of China (English)

    陈梅

    2016-01-01

    Objective: To explore the clinical effect of emergency measures and nursing intervention measures on the implementation of neonatal intensive care for patients with severe pneumonia.Methods:24 cases of severe pneumonia in our hospital were analyzed and discussed, and the comparison of the physical recovery before and after treatment was carried out in 24 cases.Results:Compared with before treatment, the treatment of severe pneumonia in neonatal clinical recovery is very obvious.Conclusion:After a period of time of emergency treatment and nursing intervention treatment, 24 cases of severe pneumonia of newborn the clinical recovery effect is very obvious. Therefore, the emergency measures and nursing intervention in clinical treatment of severe pneumonia in neonates with widely popularized significance.%目的:探讨临床上对患有重症肺炎新生儿实施急救措施和护理干预措施的效果。方法:采取随机实验法,以我院收治的24例重症肺炎的新生儿为研究对象进行分析讨论,对这24例新生儿进行治疗前和治疗后的身体恢复情况的比较。结果:相比于治疗前,治疗后的重症肺炎的新生儿临床恢复效果非常明显。结论:经过一段时间的急救和护理干预的治疗后,这24例重症肺炎的新生儿的临床恢复效果非常明显。所以,急救措施和护理干预措施在临床上治疗重症肺炎的新生儿具有广泛的推广意义。

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

    Directory of Open Access Journals (Sweden)

    Francesco Tonelli

    2012-01-01

    Full Text Available 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.

  6. Neonatal infectious diseases: evaluation of neonatal sepsis.

    Science.gov (United States)

    Camacho-Gonzalez, Andres; Spearman, Paul W; Stoll, Barbara J

    2013-04-01

    Neonatal sepsis remains a feared cause of morbidity and mortality in the neonatal period. Maternal, neonatal, and environmental factors are associated with risk of infection, and a combination of prevention strategies, judicious neonatal evaluation, and early initiation of therapy are required to prevent adverse outcomes. This article reviews recent trends in epidemiology and provides an update on risk factors, diagnostic methods, and management of neonatal sepsis. Copyright © 2013 Elsevier Inc. All rights reserved.

  7. Pathologic femur fracture due to a brown tumor in a patient with secondary hyperparathyroidism and vitamin D-resistant rickets.

    Science.gov (United States)

    Wallace, Eric; Day, Matthew; Fadare, Oluwole; Schaefer, Heidi

    2013-02-01

    Vitamin D-resistant rickets is the common clinical outcome of multiple genetic mutations that alter the regulation of phosphorus and vitamin D metabolism, mainly through their effects on fibroblast growth factor 23 (FGF-23). These diseases typically present in childhood with the classic physical examination finding of nutritional rickets, such as genu varum/valgum and rachitic rosary. Treatment, which is aimed at improving severe bone disease with vitamin D and phosphorus supplementation, can cause secondary hyperparathyroidism and/or kidney failure from nephrocalcinosis over the life of the patient. Although FGF-23 has been shown to downregulate parathyroid hormone in vitro, its effect on parathyroid secretion in disease states such as chronic kidney disease and X-linked hypophosphatemic rickets is unclear because elevations in FGF-23 and parathyroid hormone levels characterize both of these disease states. We describe a case of vitamin D-resistant rickets that presented with a femur fracture through a brown tumor. Radiographs show the combination of severe bony abnormalities associated with both long-standing hyperparathyroidism and vitamin D-resistant rickets.

  8. Acute kidney injury in asphyxiated neonates

    Directory of Open Access Journals (Sweden)

    Roy Amardiyanto

    2013-07-01

    Full Text Available Background Asphyxia neonatorum may result in multiorgan dysfunction including renal involvement. There is no consensus on the determination of acute kidney injury (AKI in neonates making establishment of the diagnosis and its management becomes difficult. The Acute Kidney Injury Network (AKIN recommends AKI criteria based on increased serum creatinine level and reduced urine output. Objectives To identify the prevalence of AKI in asphyxiated neonates using the AKIN criteria, to compare the difference of AKI stages, and the glomerular filtration rates (GFR between moderate and severe asphyxia. Methods This was a cross-sectional analytical study conducted between July 2012 and January 2013. Subjects were all asphyxiated neonates (Apgar score 35 weeks delivered and hospitalized in Cipto Mangunkusumo Hospital and Koja District Hospital, Jakarta, Indonesia. Glomerular filtration rate was calculated using the components of urine creatinine, serum creatinine, and urine output; while AKI stages were determined according to AKIN criteria. Urinary output was measured via urethral catheterization. Results Of 94 subjects, there were 70 neonates with moderate and 24 neonates with severe asphyxia, with the prevalence of AKI was 63%. Twenty one out of 24 neonates with severe asphyxia experienced AKI, while neonates with moderate asphyxia who experienced AKI was 38 out of 70 subjects (54%. Two third of neonates with severe asphyxia who experienced AKI had stage 3 of AKI. More severe AKI stages and lower median GFR were found in neonates with severe compared to moderate asphyxia (P<0.001. Conclusion The prevalence of AKI in neonatal asphyxia is high (63%. The more severe degree of neonatal asphyxia, the more severe AKI stage and the lower median GFR. [Paediatr Indones. 2013;53:232-8.].

  9. [Surgical and non-surgical management of primary hyperparathyroidism:How do calcimimetics work?

    Science.gov (United States)

    Takeuchi, Yasuhiro

    2017-01-01

    Primary hyperparathyroidism is a common endocrine disease. The first line therapy for the disease is surgical removal of affected parathyroid gland(s). Other therapeutic options with medication are needed to be established, because many of patients with primary hyperparathyroidism have few or no symptoms and are expected to have a long life expectancy without surgery. Cinacalcet as a calcimimetic, bisphosphonates and denosumab are promising candidates for medical management of the disease. Effectiveness and efficiency of these drugs for patients with primary hyperparathyroidism is to be evaluated in comparison with surgical treatment.

  10. Brown tumor of the mandible as first manifestation of primary hyperparathyroidism: diagnosis and treatment.

    Science.gov (United States)

    Fernández-Sanromán, Jacinto; Antón-Badiola, Iosu María; Costas-López, Alberto

    2005-01-01

    Brown tumor is one of the lesions that develop in patients with hyperparathyroidism. Any of the skeletal bones can be affected including the cranio-maxillofacial ones. Most of the times the brown tumor appears after a final diagnosis of hyperparathyroidism is made. However brown tumor can be the first clinical sign of the disease. A clinical case in which a brown tumor located in the anterior part of the mandible appears as the first sign of primary hyperparathyroidism is presented. The possible differential clinical diagnosis and the recommended treatments are revised.

  11. Brown tumor of the maxilla in patient with secondary hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Jović Nebojša

    2004-01-01

    Full Text Available Brown tumor or parathyroid osteopathy is a kind of bony lesion caused by hyperparathyroidism. It appears as an expansive osteolytic lesion mostly in mandible, ribs, pelvis and femur, but rarely in the upper jaw. Bone resorption is the result of osteoclastic activity due to an increased activity of parathyroid hormone. A 25-years-old male patient was operated on due to clinicaly and radiographicaly obvious maxillary tumor and increased values of parathyroid hormon (PTH - 1 050 ng/l. The level of calcium in blood was normal (Ca 2.34 mEq/L. The patient was dialyzed for years because of the chronic renal failure. Histopathologic analysis confirmed brown tumor, that appeared as bony lesion of secondary hyperparathyroidism due to the chronic renal failure. The operation of the upper jaw had been performed before parathyroidectomy, due to an excessive growth of tumor followed by heavy epistaxes. The subsequent parathyroidectomy was followed by the regression of remaining bony lesions.

  12. CINACALCET IMPROVES BONE DENSITY IN POST KIDNEY TRANSPLANT HYPERPARATHYROIDISM

    Science.gov (United States)

    Cho, ME; Duan, Z; Chamberlain, CE; Reynolds, JC; Ring, MS; Wright, EC; Mannon, RB

    2010-01-01

    The recent availability of cinacalcet has provided a possible alternative to parathyroidectomy in kidney transplant patients with persistent hyperparathyroidism, but its effect on bone mass density (BMD) is unknown. From our database containing 163 kidney transplants performed at our center from 1999-2007, we compared recipients who received cinacalcet for persistent hypercalcemia and hyperparathyroidism following renal tx (n=8; CIN) with up to 2 other post tx patients matched for age, sex, race, and graft function (n=15; CON). The outcome of the study was BMD changes from baseline to 12, 24, and 36 months post renal tx. Repeated Measures Mixed model was used to assess the difference of BMD change between two groups. Cinacalcet therapy was started at a median of 9 months (range; 1, 24 months) post tx with a mean dose 56±29 mg/d (mean duration; 1.6 years, range; 1, 2.1). Cinacalcet therapy was associated with significant reduction of serum calcium compared to control. Cinacalcet therapy was associated with greater BMD increase at the hip over the 36-month post transplant period. Cinacalcet was well tolerated. Our results suggest that cinacalcet may have a small but favorable effect on bone density following kidney transplantation. PMID:21094814

  13. Cerebral aspects of neonatal extracorporeal membrane oxygenation: a review.

    NARCIS (Netherlands)

    Mol, A.C. de; Liem, K.D.; Heijst, A.F.J. van

    2013-01-01

    Background: Neonatal extracorporeal membrane oxygenation (ECMO) is a lifesaving therapeutic approach in newborns suffering from severe, but potentially reversible, respiratory insufficiency, mostly complicated by neonatal persistent pulmonary hypertension. However, cerebral damage, intracerebral hem

  14. Supra-treatment threshold neonatal jaundice: Incidence in HIV ...

    African Journals Online (AJOL)

    Introduction. Jaundice is the yellowish pigmentation of the skin, sclera, and mucous ... We carried out a study to determine the incidence of severe and clinical neonatal ... of the non-exposed neonates had clinical jaundice of bilirubin levels.

  15. Neonatal Kraniefraktur

    DEFF Research Database (Denmark)

    Johannesen, Katrine Marie Harries; Stantchev, Hristo

    2015-01-01

    During the latest decades the incidence of birth traumas has decreased significantly. Even so the traumas still contribute to an increased mortality and morbidity. We present a case of spontaneous neonatal skull fracture following a normal vaginal delivery. Abnormal facial structure was seen......, and the fracture was identified with an MRI. The fractures healed without neurosurgical intervention. Case reports show that even in uncomplicated vaginal deliveries skull fractures can be seen and should be suspected in children with facial abnormalities....

  16. Neonatal Listeriosis

    Directory of Open Access Journals (Sweden)

    Shih-Yu Chen

    2007-01-01

    Full Text Available In Western developed countries, Listeria monocytogenes is not an uncommon pathogen in neonates. However, neonatal listeriosis has rarely been reported in Taiwan. We describe two cases collected from a single medical institute between 1990 and 2005. Case 1 was a male premature baby weighing 1558 g with a gestational age of 31 weeks whose mother had fever with chills 3 days prior to delivery. Generalized maculopapular rash was found after delivery and subtle seizure developed. Both blood and cerebrospinal fluid culture collected on the 1st day yielded L. monocytogenes. In addition, he had ventriculitis complicated with hydrocephalus. Neurologic development was normal over 1 year of follow-up after ventriculoperitoneal shunt operation. Case 2 was a 28-weeks' gestation male premature baby weighing 1180 g. Endotracheal intubation and ventilator support were provided after delivery due to respiratory distress. Blood culture yielded L. monocyto-genes. Cerebrospinal fluid showed pleocytosis but the culture was negative. Brain ultrasonography showed ventriculitis. Sudden deterioration with cyanosis and bradycardia developed on the 8th day and he died on the same day. Neonatal listeriosis is uncommon in Taiwan, but has significant mortality and morbidity. Early diagnosis of perinatal infection relies on high index of suspicion in perinatal health care professionals. [J Formos Med Assoc 2007;106(2:161-164

  17. Neonatal hemophilia: a rare presentation

    Directory of Open Access Journals (Sweden)

    Nuno Ferreira

    2015-12-01

    Full Text Available Hemophilia A is a X-linked hereditary condition that lead to decreased factor VIII activity, occurs mainly in males. Decreased factor VIII activity leads to increased risk of bleeding events. During neonatal period, diagnosis is made after post-partum bleeding complication or unexpected bleeding after medical procedures. Subgaleal hemorrhage during neonatal period is a rare, severe extracranial bleeding with high mortality and usually related to traumatic labor or coagulation disorders. Subgaleal hemorrhage complications result from massive bleeding. We present a neonate with unremarkable family history and uneventful pregnancy with a vaginal delivery with no instrumentation, presenting with severe subgaleal bleeding at 52 hours of life. Aggressive support measures were implemented and bleeding managed. The unexpected bleeding lead to a coagulation study and the diagnosis of severe hemophilia A. There were no known sequelae. This case shows a rare hemophilia presentation reflecting the importance of coagulation studies when faced with unexplained severe bleeding.

  18. Neonatal pulmonary artery thrombosis

    Directory of Open Access Journals (Sweden)

    Mangesh Jadhav

    2012-01-01

    Full Text Available Pulmonary artery thrombosis in neonates is a rare entity. We describe two neonates with this diagnosis; their presentation, evaluation, and management. These cases highlight the importance of this differential diagnosis when evaluating the cyanotic neonate.

  19. Neonatal circumcision.

    Science.gov (United States)

    Lerman, S E; Liao, J C

    2001-12-01

    The merits of neonatal circumcision continue to be debated hotly. Some argue that circumcision is a "uniquely American medical enigma." Most of the world's male population remains uncircumcised; however, most boys born in the United States continue to undergo neonatal circumcision. Review of existing literature supports that most children who are uncircumcised do well from a medical standpoint and, thus, the question of whether US health care practitioners are subjecting neonates to an unnecessary surgical procedure remains. The medical benefits of circumcision are multiple, but most are small. The clearest medical benefit of circumcision is the relative reduction in the risk for a UTI, especially in early infancy. Although this risk [figure: see text] is real, the absolute numbers are small (risk ranges from 1 in 100 to 1 in 1000), and one investigator has estimated that it may take approximately 80 neonatal circumcisions to prevent one UTI. In the case of a patient with known urologic abnormalities that predispose to UTI, neonatal circumcision has a clearer role in terms of medical benefit to the patient. Most of the other medical benefits of circumcision probably can be realized without circumcision as long as access to clean water and proper penile hygiene are achieved. Proper penile hygiene should all but eliminate the risk for foreskin-related medical problems that will require circumcision. Moreover, proper hygiene and access to clean water has been shown to reduce the rate of development of squamous cell carcinoma of the penis in the uncircumcised population. Proper techniques on the care of the foreskin are illustrated in the American Academy of Pediatrics pamphlet titled "How to care for the uncircumcised penis." Regarding the relationship between STDs and circumcision, patient education and the practice of low-risk sexual behavior make a far greater impact than does routine circumcision in hopes of reducing the spread of HIV and other STDs. Nevertheless

  20. Increased protein kinase A type Iα regulatory subunit expression in parathyroid gland adenomas of patients with primary hyperparathyroidism.

    Science.gov (United States)

    Hibi, Yatsuka; Kambe, Fukushi; Imai, Tsuneo; Ogawa, Kimio; Shimizu, Yoshimi; Shibata, Masahiro; Kagawa, Chikara; Mizuno, Yutaka; Ito, Asako; Iwase, Katsumi

    2013-01-01

    Protein kinase A (PKA) regulatory subunit type Iα (RIα) is a major regulatory subunit that functions as an inhibitor of PKA kinase activity. We have previously demonstrated that elevated RIα expression is associated with diffuse-to-nodular transformation of hyperplasia in parathyroid glands of renal hyperparathyroidism. The aim of the current study was to determine whether or not RIα expression is increased in adenomas of primary hyperparathyroidism (PHPT), because monoclonal proliferation has been demonstrated in both adenomas and nodular hyperplasia. Surgical specimens comprising 22 adenomas and 11 normal glands, obtained from 22 patients with PHPT, were analyzed. Western blot and immunohistochemical analyses were employed to evaluate RIα expression. PKA activities were determined in several adenomas highly expressing RIα. RIα expression was also separately evaluated in chief and oxyphilic cells using the "Allred score" system. Expression of proliferating cell nuclear antigen (PCNA), a proliferation marker, was also immunohistochemically examined. Western blot analysis revealed that 5 out of 8 adenomas highly expressed RIα, compared with normal glands. PKA activity in adenomas was significantly less than in normal glands. Immunohistochemical analysis further demonstrated high expression of RIα in 20 out of 22 adenomas. In adenomas, the greater RIα expression and more PCNA positive cells were observed in both chief and oxyphilic cells. The present study suggested that high RIα expression could contribute to monoclonal proliferation of parathyroid cells by impairing the cAMP/PKA signaling pathway.

  1. The utility of 99mTc-sestamibi scintigraphy in the localisation of parathyroid adenomas in primary hyperparathyroidism.

    LENUS (Irish Health Repository)

    Glynn, N

    2012-02-01

    BACKGROUND: There are conflicting data in the literature about the sensitivity of sestamibi scintigraphy in parathyroid tumour localisation in primary hyperparathyroidism (PHPT). AIM: We aimed to evaluate the overall sensitivity of this modality in parathyroid tumour localisation and to determine clinical and biochemical factors which influence sensitivity of this method. METHODS: We performed a retrospective review of 57 patients with a biochemical diagnosis of PHPT who had sestamibi scintigraphy performed. RESULTS: The sensitivity of sestamibi scanning was 56% in whole group and 63% in those without nodular thyroid disease. Among the patients with confirmed single gland disease (biochemical cure after surgical removal of a single adenoma), sensitivity was 71%. A positive scan was associated with younger age, greater adenoma weight and higher pre-operative serum calcium. Concordance between the sestamibi and neck ultrasonography was 92% accurate in pre-operative tumour localisation. CONCLUSION: Sestamibi scintigraphy was more likely to be positive in younger patients without nodular thyroid disease who have larger parathyroid adenomas with more severe hyperparathyroidism.

  2. Concomitant Graves' disease and primary hyperparathyroidism: the first case report in mainland of China and literature review

    Institute of Scientific and Technical Information of China (English)

    肖海鹏; 余斌杰; 王深明; 陈国锐

    2002-01-01

    @@ Concurrent Graves' disease and primary hyperparathyroidism in the same patient is rare, probably accounts for hypercalcemia in no more than 1 percent of thyrotoxic patients.1 Hypercalcemia may be noted during the course of hyperthyroidism in as many as 22 percent of cases.2 The cause of hypercalcemia in a thyrotoxic patient might be due to the activation of osteoclastic bone resorption3,4 by the excess thyroid hormone, as the severity of hyperthyroidism correlates positively with osteoclastic activity in trabecular and cortical bone.5 In 1936, Noble JF et al reported the first case in the world.6 To our knowledge, only 49 such cases have been described in the literature until the year of 1989.7 No case has been reported again afterward. The occurrence of hypercalcemia in a patient with hyperthyroidism may present a challenging diagnostic problem. In this communication, we described the first case in mainland of China with hypercalcemia caused by concurrent hyperthyroidism and primary hyperparathyroidism, and the clinical and laboratory characteristics were studied before and after therapy with anti-thyroid medication.

  3. Abnormal muscle and hematopoietic gene expression may be important for clinical morbidity in primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Reppe, Sjur; Stilgren, Lis; Abrahamsen, Bo

    2007-01-01

    In primary hyperparathyroidism (PHPT), excess PTH secretion by adenomatous or hyperplastic parathyroid glands leads to elevated serum [Ca(2+)]. Patients present complex symptoms of muscular fatigue, various neuropsychiatric, neuromuscular, and cardiovascular manifestations, and, in advanced disease...

  4. Image guided, minimally invasive adenomectomy for solitary gland disease in primary hyperparathyroidism

    NARCIS (Netherlands)

    Smit, Pieter Casper

    2001-01-01

    Introduction: Since the introduction in the 1970s of the unilateral approach in surgery for primary hyperparathyroidism by Wang, authors have increasingly been recommending limited forms of parathyroid surgery. Although unilateral explorations reduce operation time and admission days, decrease

  5. Radicular lower extremity pain as the first symptom of primary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Mustonen, Antti O.T.; Kiuru, Martti J.; Koskinen, Seppo K. [Toolo Trauma Center, Helsinki University Central Hospital, Topeliuksenkatu 5, 00029, Helsinki (Finland); Stahls, Anders; Bohling, Tom [Department of Pathology, Haartman Institute, University of Helsinki, 00014, Helsinki (Finland); Kivioja, Aarne [Department of Orthopedics and Traumatology, Helsinki University Central Hospital, 00029, Helsinki (Finland)

    2004-08-01

    Clinical symptoms of hyperparathyroidism are generally nausea, vomiting, fatigue, constipation, and hypotonicity of the muscles and ligaments; bone pain and tenderness are also seen but are more common in secondary hyperparathyroidism. We report a histologically confirmed case of a 28-year-old man whose sole symptom of primary hyperparathyroidism was lower extremity radicular pain due to a vertebral brown tumor. Magnetic resonance imaging demonstrated brown tumor to be hyperintense on T2-weighted and slightly hypointense on T1-weighted sequences; it showed intense contrast enhancement with gadolinium. Because brown tumors usually contain hemosiderin a short T2 should have been expected, but this was not seen in our case. Healing resulted in decreasing contrast enhancement on T1-weighted sequences and increasingly short T2. To our knowledge, this is the first report of a lumbar vertebral brown tumor associated with primary hyperparathyroidism. (orig.)

  6. A randomized study evaluating cinacalcet to treat hypercalcemia in renal transplant recipients with persistent hyperparathyroidism

    DEFF Research Database (Denmark)

    Evenepoel, P.; Cooper, K.; Holdaas, H.

    2014-01-01

    Persistent hyperparathyroidism (HPT) after kidney transplantation (KTx) is associated with hypercalcemia, hypophosphatemia and abnormally high levels of parathyroid hormone (PTH). In this randomized trial, cinacalcet was compared to placebo for the treatment of hypercalcemia in adult patients...

  7. Surgeon-Performed Ultrasound as Preoperative Localization Study in Patients with Primary Hyperparathyroidism

    NARCIS (Netherlands)

    T.M. van Ginhoven (Tessa); A.N. Morks; T. Schepers (Tim); P.W. de Graaf; P.C. Smits (Pieter)

    2011-01-01

    textabstractBackground: Minimally invasive parathyroidectomy is the treatment of choice for single-gland primary hyperparathyroidism. However, the exact location of the abnormal gland has to be established. Sestamibi scintigraphy, computed tomography and ultrasound (US) are commonly used modalities.

  8. Surgeon-Performed Ultrasound as Preoperative Localization Study in Patients with Primary Hyperparathyroidism

    NARCIS (Netherlands)

    van Ginhoven, T. M.; Morks, A. N.; Schepers, T.; de Graaf, P. W.; Smit, P. C.

    2011-01-01

    Background: Minimally invasive parathyroidectomy is the treatment of choice for single-gland primary hyperparathyroidism. However, the exact location of the abnormal gland has to be established. Sestamibi scintigraphy, computed tomography and ultrasound (US) are commonly used modalities. We describe

  9. "Porcelain heart" cardiomyopathy secondary to hyperparathyroidism: radiographic, echocardiographic, and cardiac CT appearances.

    LENUS (Irish Health Repository)

    Freeman, James

    2010-11-01

    We report the radiographic, echocardiographic and cardiac CT appearances of \\'porcelain heart\\' in an 85-year-old woman who presented with progressive heart failure. The extensive myocardial calcification was secondary to hyperparathyroidism with renal failure.

  10. "Porcelain heart" cardiomyopathy secondary to hyperparathyroidism: radiographic, echocardiographic, and cardiac CT appearances.

    LENUS (Irish Health Repository)

    Freeman, James

    2012-02-01

    We report the radiographic, echocardiographic and cardiac CT appearances of \\'porcelain heart\\' in an 85-year-old woman who presented with progressive heart failure. The extensive myocardial calcification was secondary to hyperparathyroidism with renal failure.

  11. Nontyphoidal salmonella urinary tract infection in a case of hyperparathyroidism and nephrocalcinosis.

    Science.gov (United States)

    Chen, C-P; Shi, Z-Y; Chen, C-H; Chen, W-M; Lin, Y-H; Tsai, C-A; Lin, S-P; Huang, S-R; Liu, P-Y

    2014-01-01

    Nontyphoidal Salmonella infections often present with self-limited gastroenteritis. Extraintestinal focal infections are uncommon but have high mortality and morbidity. Urinary tract infection caused by nontyphoidal Salmonella is usually associated with structural abnormalities of the urinary tract. Nephrocalcinosis and nephrolithiasis are the major risk factors. Although primary hyperparathyroidism has been reported to increase the risk of nephrocalcinosis and nephrolithiasis, little is known about the association between hyperparathyroidism and Salmonella urinary tract infection. We report the case of a 37-year old man who had a history of primary hyperparathyroidism and bilateral nephrocalcinosis and who developed urinary tract infection. Salmonella Group D was isolated from his urine specimen. Salmonella should be considered as a possible causality organism in patients with primary hyperparathyroidism and nephrocalcinosis who develop urinary tract infection. These patients need to be aware of the potential risks associated with salmonellosis.

  12. Image guided, minimally invasive adenomectomy for solitary gland disease in primary hyperparathyroidism

    NARCIS (Netherlands)

    Smit, Pieter Casper

    2001-01-01

    Introduction: Since the introduction in the 1970s of the unilateral approach in surgery for primary hyperparathyroidism by Wang, authors have increasingly been recommending limited forms of parathyroid surgery. Although unilateral explorations reduce operation time and admission days, decrease opera

  13. Comparative characteristics of primary hyperparathyroidism in pediatric and young adult patients.

    Science.gov (United States)

    Nicholson, Kristina J; McCoy, Kelly L; Witchel, Selma F; Stang, Michael T; Carty, Sally E; Yip, Linwah

    2016-10-01

    Primary hyperparathyroidism is rare in pediatric patients. Our study aim was to compare primary hyperparathyroidism in pediatric (pediatric and 87 young adult patients. Presenting symptoms, operative data, and postoperative course were compared for patients age 0-19 years and 20-29 years. Sporadic primary hyperparathyroidism was present in 81.7% and occurred less often in pediatric patients than young adult patients (74.4% vs 86.2%, P = .12). Among patients with hereditary primary hyperparathyroidism, multiple endocrine neoplasia type 1 was the most common type. Multiglandular disease was common in both pediatric (30.7%) and young adult (21.8%) patients. Following parathyroidectomy, 3 (2.3%) patients had permanent hypoparathyroidism and none had permanent recurrent laryngeal nerve paralysis. Biochemical cure at 6 months was equally likely in pediatric and young adult patients (97.1% vs 93.6%, P = .44) with comparable follow-up (78.4 months vs 69.1 months, P = .66) and rates of recurrent disease (5.9% vs 10.3%, P = .46). Recurrence was due to multiple endocrine neoplasia 1-related primary hyperparathyroidism in all cases. Although primary hyperparathyroidism is sporadic in most patients multiple endocrine neoplasia type 1-associated primary hyperparathyroidism (23%). Parathyroidectomy for primary hyperparathyroidism can be performed safely in pediatric patients with a high rate of cure. Follow-up for patients with hereditary disease is necessary. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. PRIMARY HYPERPARATHYROIDISM AT THE BACKGROUND OF PARATHYROID GLAND HYPERPLASIA. PECULIARITIES AND DIFFICULTIES OF DIAGNOSTICS

    Directory of Open Access Journals (Sweden)

    L. P. Yakovleva

    2015-01-01

    Full Text Available The article uses a clinical case of primary hyperparathyroidism in young patient with hyperplastic parathyroid gland localization in tissues of thyroid gland as an example to study the matters of cytological, histological, clinical and laboratory diagnostics of such pathology. It provides a literature reference on the difficulties of establishing a cytological and histological diagnoses, sets out the peculiarities of morphological pattern, discusses the tactical matters of primary hyperparathyroidism management, indications for surgical treatment and case follow-up of patients.

  15. Primary hyperparathyroidism presenting as recurrent acute pancreatitis: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Raiz A Misgar

    2011-01-01

    Full Text Available The association between pancreatitis and primary hyperparathyroidism (PHPT is controversial. We report a 32-year-old man who presented with recurrent episodes of acute pancreatitis. Primary hyperparathyroidism was diagnosed after the fourth episode of pancreatitis. He had no additional risk factors for pancreatitis. Eighteen months after successful parathyroid surgery, there has been no recurrence of abdominal pain and his serum calcium is within the normal range.

  16. Tentorial and dural calcification with tertiary hyperparathyroidism: a rare entity in chronic renal failure

    Energy Technology Data Exchange (ETDEWEB)

    Dorenbeck, U.; Bretschneider, T.; Feuerbach, S. [Department of Diagnostic Radiology, University Hospital of Regensburg, Franz-Josef-Strauss-Allee 11, 93042 Regensburg (Germany); Leingaertner, T.; Kraemer, B.K. [Department of Internal Medicine II, University Hospital of Regensburg, Franz-Josef-Strauss-Allee 11, 93042 Regensburg (Germany)

    2002-07-01

    A case of rare calcification of the tentorium cerebelli, the thoracal dura mater of the spine, and the sclera in an adult male patient with tertiary hyperparathyroidism is presented. The often reported feature in the skull is that of a combination of osteopenia and osteosclerosis giving a granular bone texture. Extensive dura calcification with this condition has very rarely been reported. It is the aim of this paper to document the latter in a patient with chronic renal failure and tertiary hyperparathyroidism. (orig.)

  17. Image guided, minimally invasive adenomectomy for solitary gland disease in primary hyperparathyroidism

    OpenAIRE

    Smit, Pieter Casper

    2001-01-01

    Introduction: Since the introduction in the 1970s of the unilateral approach in surgery for primary hyperparathyroidism by Wang, authors have increasingly been recommending limited forms of parathyroid surgery. Although unilateral explorations reduce operation time and admission days, decrease operative risk and give better cosmetic results, the debate about the best surgical treatment for primary hyperparathyroidism has never been settled. ‘Bilateralists’ oppose less invasive approaches beca...

  18. [A patient with coexistence of primary hyperparathyroidism, Marfan's syndrome and von Willebrand's disease].

    Science.gov (United States)

    Bednarek-Tupikowska, Grazyna; Rakowska-Chort, Anna; Adamarczuk-Janczyszyn, Maria

    2008-01-01

    We presented a case of a 58 year old women suffering from three diseases: primary hyperparathyroidism, Marfan's syndrome and von Willebrand's disease. The coexistence of these diseases is not mentioned in medical literature. Because of the coexistence of Marfan's syndrome with primary hyperparathyroidism, the examinations of other endocrinopathy including multiple endocrine neoplasia (MEN). Their coexistence was not confirmed at the present time. In this paper the new views on both patogenesis and treatment of the mentioned diseases were included.

  19. High frequency of asymptomatic hyperparathyroidism in patients with fibromyalgia: random association or misdiagnosis?

    Science.gov (United States)

    Costa, Juliana Maria de Freitas Trindade; Ranzolin, Aline; Neto, Cláudio Antônio da Costa; Marques, Claudia Diniz Lopes; Duarte, Angela Luzia Branco Pinto

    2016-03-22

    Fibromyalgia (FM) and hyperparathyroidism may present similar symptoms (musculoskeletal pain, cognitive disorders, insomnia, depression and anxiety), causing diagnostic confusion. To determine the frequency of asymptomatic hyperparathyroidism in a sample of patients with FM and to evaluate the association of laboratory abnormalities to clinical symptoms. Cross-sectional study with 100 women with FM and 57 healthy women (control group). Parathyroid hormone (PTH), calcium and albumin levels were accessed, as well as symptoms in the FM group. In FM group, mean serum calcium (9.6±0.98mg/dL) and PTH (57.06±68,98 pg/mL) values were considered normal, although PTH levels had been significantly higher than in the control group (37.12±19.02 pg/mL; p=0.001). Hypercalcemic hyperparathyroidism was diagnosed in 6% of patients with FM, and 17% of these women exhibited only high levels of PTH, featuring a normocalcemic hyperparathyroidism, with higher frequencies than those expected for their age. There was no significant association between hyperparathyroidism and FM symptoms, except for epigastric pain, which was more frequent in the group of patients concomitantly with both diseases (p=0.012). A high frequency of hyperparathyroidism was noted in women with FM versus the general population. Normocalcemic hyperparathyroidism was also more frequent in patients with FM. Longitudinal studies with greater number of patients are needed to assess whether this is an association by chance only, if the increased serum levels of PTH are part of FM pathophysiology, or even if these would not be cases of FM, but of hyperparathyroidism. Copyright © 2015. Published by Elsevier Editora Ltda.

  20. Primary hyperparathyroidism associated with a giant cell tumor: One case in the distal radius.

    Science.gov (United States)

    Ouzaa, M R; Bennis, A; Iken, M; Abouzzahir, A; Boussouga, M; Jaafar, A

    2015-10-01

    Hyperparathyroidism can present itself as brown tumors (or osteolytic expansive lesions) that usually disappear after normalization of calcium and phosphate levels. It rarely occurs simultaneously with a giant cell tumor. The authors report one case of a localized form at the distal radius in a patient being followed for primary hyperparathyroidism. The diagnostic challenges related to the clinical and radiological similarities of these two pathological entities are discussed, as they can lead to delays in therapeutic management.

  1. High frequency of asymptomatic hyperparathyroidism in patients with fibromyalgia: random association or misdiagnosis?

    Directory of Open Access Journals (Sweden)

    Juliana Maria de Freitas Trindade Costa

    Full Text Available ABSTRACT Fibromyalgia (FM and hyperparathyroidism may present similar symptoms (musculoskeletal pain, cognitive disorders, insomnia, depression and anxiety, causing diagnostic confusion. Objectives: To determine the frequency of asymptomatic hyperparathyroidism in a sample of patients with FM and to evaluate the association of laboratory abnormalities to clinical symptoms. Methods: Cross-sectional study with 100 women with FM and 57 healthy women (comparison group. Parathyroid hormone (PTH, calcium and albumin levels were accessed, as well as symptoms in the FM group. Results: In FM group, mean serum calcium (9.6 ± 0.98 mg/dL and PTH (57.06 ± 68.98 pg/mL values were considered normal, although PTH levels had been significantly higher than in the comparison group (37.12 ± 19.02 pg/mL; p = 0.001. Hypercalcemic hyperparathyroidism was diagnosed in 6% of patients with FM, and 17% of these women exhibited only high levels of PTH, featuring a normocalcemic hyperparathyroidism, with higher frequencies than those expected for their age. There was no significant association between hyperparathyroidism and FM symptoms, except for epigastric pain, which was more frequent in the group of patients concomitantly with both diseases (p = 0.012. Conclusions: A high frequency of hyperparathyroidism was noted in women with FM versus the general population. Normocalcemic hyperparathyroidism was also more frequent in patients with FM. Longitudinal studies with greater number of patients are needed to assess whether this is an association by chance only, if the increased serum levels of PTH are part of FM pathophysiology, or even if these would not be cases of FM, but of hyperparathyroidism.

  2. Primary Hyperparathyroidism-A Review of this Disorder and an Interesting Case Treated with Cinacalcet

    Directory of Open Access Journals (Sweden)

    Abdul Rafi Mohammed

    2009-06-01

    Full Text Available Primary hyperparathyroidism is not an uncommon disorder and there can be a delay in the diagnosis as it remains mostly asymptomatic. We discuss about this disorder and review the literature. Treatment options which include both surgical and medical therapy are also discussed. We also report an interesting case of primary hyperparathyroidism which was treated with Cinacalcet. Turk Jem 2009; 13: 37-9

  3. Amelioration of Sickle Cell Pain after Parathyroidectomy in Two Patients with Concurrent Hyperparathyroidism: An Interesting Finding

    Directory of Open Access Journals (Sweden)

    John Muthu

    2016-01-01

    Full Text Available Patients with sickle cell disease have high morbidity and healthcare utilization due to repeated painful crises. Some coexisting conditions which cause pain similar to sickle cell disease may go undiagnosed in these patients. We report two adults with concurrent hyperparathyroidism who experienced significant improvement in sickle cell pain following parathyroidectomy thereby pointing to hyperparathyroidism as the principal causative factor for their pain. Meticulous evaluation for parathyroid disorders can be rewarding in sickle cell disease.

  4. Increased tissue-type plasminogen activator antigen release is not accompanied by increased systemic fibrinolytic activity in severe neonatal respiratory distress syndrome

    NARCIS (Netherlands)

    Brus, F; Oetomo, SB; Schieving, J; Groothuis, E; Okken, A; van Oeveren, W

    1999-01-01

    Intravascular and intraalveolar fibrin depositions in preterm infants with severe respiratory distress syndrome (RDS) have been attributed to activation of clotting. We questioned whether in the face of activated clotting, Fibrinolysis is sufficient in these infants. We found, in infants with severe

  5. Neonatal compartment syndrome.

    Science.gov (United States)

    Martin, B; Treharne, L

    2016-09-01

    A term neonate was born with a grossly swollen and discoloured left hand and forearm. He was transferred from the local hospital to the plastic surgical unit, where a diagnosis of compartment syndrome was made and he underwent emergency forearm fasciotomies at six hours of age. Following serial debridements of necrotic tissue, he underwent split-thickness skin grafting of the resultant defects of his forearm, hand and digits. At the clinic follow-up appointment two months after the procedure, he was found to have developed severe flexion contractures despite regular outpatient hand therapy and splintage. He has had further reconstruction with contracture release, use of artificial dermal matrix, and K-wire fixation of the thumb and wrist. Despite this, the long term outcome is likely to be an arm with poor function. The key learning point from this case is that despite prompt transfer, diagnosis and appropriate surgical management, the outcome for neonatal compartment syndrome may still be poor.

  6. OUTCOME OF NEONATES WITH THROMBOCYTOPENIA

    Directory of Open Access Journals (Sweden)

    Sharangouda

    2014-04-01

    Full Text Available OBJECTIVE: To determine etiology, onset, clinical features and outcome of neonates with thrombocytopenia. METHODS: 140 neonates having bleeding or having platelet count (<1.5lakhs/µl were selected from those admitted to NICU’S attached to MR Medical College, Gulbarga. Initial platelet count was done on admission and counts were repeated 12 hours after any therapeutic intervention. OBSERVATION AND RESULTS: Severe thrombocytopenia (<50000/µl was present in 8.5%, moderate (50, 000-1, 00, 000/µl in 17%. Majority (45.33% were preterm and the major cause was sepsis in 51.3%.Mucosal bleed was the most common presentation. Mortality was 37% in severe and 3.9% in moderate thrombocytopenia group. CONCLUSION: Significant association is observed with maternal PIH, Late onset sepsis, NEC and sepsis with DIC .Prematurity, IUGR, Birth asphyxia were common associated morbidities. Severe thrombocytopenia in sick neonates, in NICU, is a poor prognostic indicator.

  7. Multiple brown tumors of the jaws in primary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Kyoung A; Koh, Kwang Joon [School of Dentisity, Chonbuk National University, Jeonju (Korea, Republic of)

    2010-09-15

    Brown tumor is usually diagnosed at the terminal stage of hyperparathyroidism. Diagnosis of this tumor is confirmed by endocrinologic investigations along with clinical and radiographic examination. Radiographical differential diagnosis of this tumor includes central giant cell granuloma, aneurysmal bone cyst, metastatic tumor, multiple myeloma, and Paget disease. This report presents a rare case of multiple brown tumors occurring at the maxilla and mandible, which was initially misdiagnosed as central giant cell granuloma. Plain radiographs demonstrated multiple well-defined multilocular radiolucency. CT images showed soft tissue mass with low attenuated lesions, perforation of the lingual cortical plate, and a heterogeneous mass at the right thyroid lobe. These findings were consistent with parathyroid adenoma. The patient had hypercalcemia, hypophosphatemia, and elevated alkaline phosphatase level. Surgical excision of the tumor was performed. No recurrence was observed during a 28-month follow-up.

  8. Usefulness of 99mTc MIBI scintigraphy in hyperparathyroidism. A retrospective analysis of the surgical patients

    Energy Technology Data Exchange (ETDEWEB)

    Nakamura, Toshiyuki; Kobayashi, Shinya; Fujimori, Minoru [Shinshu Univ., Matsumoto, Nagano (Japan). School of Medicine] [and others

    1998-10-01

    In patients who receive surgery for primary or secondary hyperparathyroidism, preoperative diagnosis of the location of the parathyroid glands is important. Ninety-nine-m Technetium methoxyisobutylisonitrile (MIBI) accumulates in the pathological parathyroid. We used MIBI scintigraphy to detect diseased parathyroid glands in 20 patients with hyperparathyroidism, and successfully located the glands in seventeen. The accuracy of MIBI scintigraphy (100%) is significantly (p<0.05) better than that of Thallium-Technetium subtraction scintigraphy (56%). In a patient with hyperparathyroidism due to an ectopic parathyroid gland, MIBI scintigraphy showed accumulation in a mediastinal gland. MIBI scintigraphy is thus useful for gland location in hyperparathyroidism. (author)

  9. Hyperparathyroidism and vitamin D deficiency after laparoscopic gastric bypass.

    Science.gov (United States)

    Clements, Ronald H; Yellumahanthi, Kishore; Wesley, Mary; Ballem, Naveen; Bland, Kirby I

    2008-06-01

    Hyperparathyroidism (HPT) can occur after gastric bypass because of the alteration in vitamin D and calcium absorption. Adequate serum vitamin D concentrations have not been clearly defined in this patient population. Vitamin D (Vit D) and parathyroid hormone (PTH) were assessed 1 year after laparoscopic gastric bypass (LGB). The prevalence of HPT and Vit D deficiency were determined and their association was evaluated using Fisher's exact test. Ninety-three patients (aged 44 +/- 1.1 years, 49.6 +/- 0.67 Kg/m2 body mass index, 79.6% female, 69.6% white) were evaluated. The prevalence of Vit D deficiency (less than 20 ng/mL) and HPT (greater than 65 pg/mL) was 23.6 per cent (n = 22) and 25.7 per cent (n = 28), respectively. Among patients with HPT, only eight of 28 (28.6%) had Vit D deficiency, and of those with Vit D deficiency, only eight of 22 (36.4%) had HPT. There was a weak inverse correlation (r = -0.37) between PTH and Vit D. Blacks are at higher risk for Vit D deficiency. There was no significant association between Vit D deficiency and HPT, Vit D deficiency and Roux limb length, or HPT and Roux limb length. After LGB, Vit D deficiency and hyperparathyroidism occur commonly. Body mass index and Roux limb length are not associated with these two conditions, but racial differences do exist. There is a weak inverse correlation between Vit D and PTH. Further research is needed to elucidate the causes, treatments, and significance of HPT after LGB.

  10. Hyperparathyroid crisis: clinical and pathologic studies of 14 patients.

    Science.gov (United States)

    Wang, C A; Guyton, S W

    1979-01-01

    A study is presented of 14 patients with hyperparathyroid crisis treated at the Massachusetts General Hospital between 1964 and 1978. These patients showed diverse clinical manifestations that were indistinguishable from those in patients with pseudohyperparathyroidism. Their symptoms varied from progressive fatigue, malaise, and weakness to those related to the gastrointestinal and urinary tracts. The one biochemical alteration commonly found among these patients was the rapid increase in the serum calcium. There was a concomitant rise in the BUN in 50% of the patients and in the creatinine in 80%. The diagnosis was established by an elevated immunoreactive parathyroid hormone (PTH) level in all eight patients (100%) who had the radioimmunoassay; by the presence of subperiosteal resorption of the phalanges in six of the eight patients (75%); and in three of four patients (75%) by the loss of the lamina dura of the teeth. The 12 patients who had surgery all survived; the two who did not died. Thirteen patients (93%) had a neoplasm--an adenoma in 12 and a carcinoma in one. One patient had hyperplasia (7%). Nine patients (64%) received hypocalcemic drug therapy. The serum calcium temporarily fell to 12 mg/100 ml in five patients (56%) but failed to budge in four (44%). Simultaneous treatment with saline infusion, furosemide and with hypocalcemic drugs over a prolonged period compounded the difficulty at operation by increasing interstitial edema. Our findings from this study show prompt surgical intervention as the ideal treatment for hyperparathyroid crisis, preferably, within 72 hours of the acute onset of symptoms. PMID:518179

  11. Laboratory technology for population-based screening for severe combined immunodeficiency in neonates: the winner is T-cell receptor excision circles.

    Science.gov (United States)

    Puck, Jennifer M

    2012-03-01

    The most profound primary immunodeficiency disease, severe combined immunodeficiency (SCID), is fatal in infancy unless affected infants are provided with an adaptive immune system through allogeneic hematopoietic cell transplantation, enzyme replacement, or gene therapy. However, most infants with SCID lack a family history or any clinical clues before the onset of infections, making this serious but treatable disease a candidate for population-based newborn screening. Of several approaches considered for SCID screening, testing for T-cell receptor excision circles (TRECs), a DNA biomarker of normal T-cell development, has proved successful. TREC numbers can be measured in DNA isolated from the dried bloodspots already routinely collected for newborn screening. Infants with low or absent TRECs can thus be identified and referred for confirmatory testing and prompt intervention. TREC testing of newborns is now being performed in several states, indicating that this addition to the newborn screening panel can be successfully integrated into state public health programs.

  12. Motion based Segmentation of Chest and Abdomen Region of Neonates

    NARCIS (Netherlands)

    Venkitaraman, A.; Makkapati, V.V.

    2015-01-01

    Respiration rate (RR) is one of the important vital signs used for clinical monitoring of neonates in intensive care units. Due to thefragile skin of the neonates, it is preferable to have monitoring systems with minimal contact with the neonate. Recently, several methods have been proposed for

  13. Motion based Segmentation of Chest and Abdomen Region of Neonates

    NARCIS (Netherlands)

    Venkitaraman, A.; Makkapati, V.V.

    2015-01-01

    Respiration rate (RR) is one of the important vital signs used for clinical monitoring of neonates in intensive care units. Due to thefragile skin of the neonates, it is preferable to have monitoring systems with minimal contact with the neonate. Recently, several methods have been proposed for con

  14. Role of pre-operative imaging using {sup 99m}Tc-MIBI and neck ultrasound in patients with secondary hyperparathyroidism who are candidates for subtotal parathyroidectomy

    Energy Technology Data Exchange (ETDEWEB)

    Fuster, David; Ortin, Jaime; Setoain, Xavier; Paredes, Pilar; Duch, Joan; Pons, Francesca [Hospital Clinic, Nuclear Medicine Department, Barcelona (Spain); Ybarra, Juan; Torregrosa, Jose-Vicente [Hospital Clinic, Renal Transplant Unit, Barcelona (Spain); Gilabert, Rosa [Hospital Clinic, Radiology Department, Barcelona (Spain)

    2006-04-15

    The purpose of this study was to assess whether pre-operative {sup 99m}Tc-methoxyisobutylisonitrile (MIBI) scintigraphy and neck ultrasound (US) are of value in improving the outcome of subtotal parathyroidectomy in patients with secondary hyperparathyroidism. Forty-eight consecutive haemodialysis patients with severe secondary hyperparathyroidism prospectively underwent ''blinded'' subtotal parathyroid surgery, with 1-year follow-up to establish cure or relapse of their secondary hyperparathyroidism. Double-phase {sup 99m}Tc-MIBI scintigraphy and neck US were performed pre-operatively in all patients. When the preserved gland showed {sup 99m}Tc-MIBI uptake or an abnormal size on US, it was considered that ''{sup 99m}Tc-MIBI advice'' and ''US advice'', respectively, had not been followed. Pre-operative and follow-up parathyroid hormone (PTH) levels were obtained in all patients. All data were evaluated on a patient by patient basis. Four parathyroid glands were identified in each patient at primary surgery, resulting in an operative success rate of 100%. Their weight ranged from 15 to 7,300 mg (mean 1,120{+-}900 mg). Nine of the 48 patients (19%) showed a recurrence of their secondary hyperparathyroidism. The recurrence rate was 2% (1/48) and 10% (5/48), respectively, when {sup 99m}Tc-MIBI and US advice was followed. The sensitivity, specificity, NPV and PPV for pre-operative imaging were 72%, 95%, 97% and 80% respectively for {sup 99m}Tc-MIBI, and 55%, 67%, 87% and 28% for US. {sup 99m}Tc-MIBI scintigraphy is a reliable non-invasive exploratory tool and its preoperative use results in a significant reduction in the number of recurrences in haemodialysis patients with secondary hyperparathyroidism who are candidates for subtotal parathyroidectomy. The use of neck US did not significantly improve the results obtained with {sup 99m}Tc-MIBI alone. (orig.)

  15. [Diagnostic approach and management of hypercalcaemia in dogs exemplary of primary hyperparathyroidism].

    Science.gov (United States)

    Ballhausen, Bianca Désirée; Wehner, Astrid; Zöllner, Martin; Hartmann, Katrin; Unterer, Stefan

    2017-03-29

    Hypercalcaemia can be caused by many different diseases. This article summarizes the causes, pathophysiologic mechanisms and diagnostic procedures as well as treatment recommendations. The main focus is on hypercalcaemia in primary hyperparathyroidism (PH), complemented by a case report. An elevated total calcium level should generally be investigated and verified by measurement of ionized calcium concentration. The further diagnostic approach depends on the phosphate level. Tumour screening, measurement of parathormone and parathromone-related protein and sonography of parathyroid glands may be necessary. If the calcium-phosphate-product exceeds 60 mg/dl, there is a risk of tissue mineralisation and a rapid treatment of hypercalcaemia is required. For acute therapy, sodium chloride infusion, furosemide and glucocorticoids can be used. Glucocorticoids should only be given after strict indication and after a definite diagnosis. For long-term management, bisphosphates, particularly alendronate, are increasingly used successfully. Causal therapy of PH can be performed by parathyreoidectomy, heat ablation or ethanol ablation. Thereafter, particularly in cases of severe preoperative hypercalcaemia, hypocalcaemia can occur. Treatment is performed using vitamin D3 (calcitriol), which may also be given preoperatively in cases of severe hypercalcaemia. A concomitant oral calcium supplementation using calcium carbonate as medication of choice is contentious. Due to a potential relapse after successful excision of the affected parathyroid gland in PH, the serum calcium level should be monitored periodically.

  16. PTH-dependence of the effectiveness of cinacalcet in hemodialysis patients with secondary hyperparathyroidism.

    Science.gov (United States)

    Akizawa, Tadao; Kurita, Noriaki; Mizobuchi, Masahide; Fukagawa, Masafumi; Onishi, Yoshihiro; Yamaguchi, Takuhiro; Ellis, Alan R; Fukuma, Shingo; Alan Brookhart, M; Hasegawa, Takeshi; Kurokawa, Kiyoshi; Fukuhara, Shunichi

    2016-04-13

    Cinacalcet lowers parathyroid hormone levels. Whether it can prolong survival of people with chronic kidney disease (CKD) complicated by secondary hyperparathyroidism (SHPT) remains controversial, in part because a recent randomized trial excluded patients with iPTH <300 pg/ml. We examined cinacalcet's effects at different iPTH levels. This was a prospective case-cohort and cohort study involving 8229 patients with CKD stage 5D requiring maintenance hemodialysis who had SHPT. We studied relationships between cinacalcet initiation and important clinical outcomes. To avoid confounding by treatment selection, we used marginal structural models, adjusting for time-dependent confounders. Over a mean of 33 months, cinacalcet was more effective in patients with more severe SHPT. In patients with iPTH ≥ 500 pg/ml, the reduction in the risk of death from any cause was about 50% (Incidence Rate Ratio [IRR] = 0.49; 95% Confidence Interval [95% CI]: 0.29-0.82). For a composite of cardiovascular hospitalization and mortality, the association was not statistically significant, but the IRR was 0.67 (95% CI: 0.43-1.06). These findings indicate that decisions about using cinacalcet should take into account the severity of SHPT.

  17. Neonatal cardiovascular physiology.

    Science.gov (United States)

    Hines, Michael H

    2013-11-01

    The pediatric surgeon deals with a large number and variety of congenital defects in neonates that frequently involve early surgical intervention and care. Because the neonatal cardiac physiology is unique, starting with the transition from fetal circulation and including differences in calcium metabolism and myocardial microscopic structure and function, it serves the pediatric surgeon well to have a sound understanding of these principles and how they directly and indirectly affect their plans and treatments. In addition, many patients will have associated congenital heart disease that can also dramatically influence not only the surgical and anesthetic care but also the timing and planning of procedures. Finally, the pediatric surgeon is often called upon to treat conditions and complications associated with complex congenital heart disease such as feeding difficulties, bowel perforations, and malrotation in heterotaxy syndromes. In this article, we will review several unique aspects of neonatal cardiac physiology along with the basic physiology of the major groups of congenital heart disease to better prepare the training and practicing pediatric surgeon for care of these complex and often fragile patients.

  18. 不同发病孕周早发型重度子痫前期母婴结局分析%Analysis of the Maternal and Neonatal Outcome of Early Onset Severe Pre-eclampsia at Different Gestational Weeks

    Institute of Scientific and Technical Information of China (English)

    刘桂玉

    2014-01-01

    Objective To investigate the af ection to the mother and neonate of early onset severe pre-eclampsia at dif erent gestational weeks.Methods 62 patients diagnosed as EOSP with the gestational weeks of 20~34 were involved in the study and divided into groups.Group A 12 patinets with the gestational week of 20~28,Group B 25 patinets with the gestational week of 28~32 and Group C 25 patinets with the gestational week of 32~34.We compared the maternal and neonatal outcome among the three groups.Results The shorter the gestational weeks of EOSP was,the higher the rate of placental abruption and fetal death was,there was significant dif erence among the outcome of the three groups( <0.05).Conclusion The prognosis of early onset of severe preeclampsia patients whose gestational weeks were more than 28 weeks were bet er.Expectant treatment can prolong the gestational week of early onset of severe pre-eclampsia patients and and it's very important to master the time and way for pregnancy termination.%目的探讨不同发病孕周早发型重度子痫前期(EOSP)对母婴结局的影响。方法62例EOSP患者,孕20~34w,其中孕20~28w12例(A组),孕28~32w25例(B组),孕32~34w25例(C组)。比较各组母婴结局。结果早发型重度子痫前期发病孕周越早,患者胎盘早剥、子痫、死胎发生率越高,母婴结局不良的几率越高。三组间差异有统计学意义,<0.05。结论孕28w后发病的早发型重度子痫前期母婴结局较好,期待治疗可以适当延长孕周,选择适宜的终止妊娠时机和方式是减少母婴并发症的有力措施。

  19. The treatment of secondary hyperparathyroidism in haemodialysis patients' refractory to alfacalcidol

    Directory of Open Access Journals (Sweden)

    L V Egshatyan

    2012-06-01

    markers decreased better in the PTx group compared to Cinacalcet group. The effectiveness and safety of Mimpara for secondary hyperparathyroidism were evaluated in dialysis patients’ refractory to alfacalcidol, which reduced the need for parathyroidectomy in patient without severe osteodystrophy.

  20. Neonatal lupus.

    Science.gov (United States)

    Robles, David T; Jaramillo, Lorena; Hornung, Robin L

    2006-12-10

    An otherwise healthy 5-week-old infant with erythematous plaques predominantly on the face and scalp presented to our dermatology clinic. The mother had been diagnosed with lupus erythematosus 2 years earlier but her disease was quiescent. Neonatal lupus is a rare condition associated with transplacental transfer of IgG anti-SSA/Ro and anti-SSB/La antibodies from the mother to the fetus. Active connective tissue disease in the mother does not have to be present and in fact is often absent. Although the cutaneous, hematologic and hepatic manifestations are transient, the potential for permanent heart block makes it necessary for this to be carefully ruled out. As in this case, the dermatologist may be the one to make the diagnosis and should be aware of the clinical presentation, work-up, and management of this important disease.

  1. A Rare Cause of Neonatal Liver Failure: Neonatal Hemochromatosis

    Directory of Open Access Journals (Sweden)

    Uluca Ü et al.

    2013-09-01

    Full Text Available Neonatal hemochromatosis (NH is a severe rare liver disease in neonatal period associated with ekstrahepatic siderosis. This disease is characterized by hepatocellular insufficiency that presented with jaundice, hypoglycemia, hypoalbuminemia, low fibrinogen levels, thrombocytopenia, anemia, direct and indirect hyperbilirubinemia from the first days of life. Herein we reported a case with Rh incompatibility whose jaundice was noted at the first day of life and referred to our hospital for exchange transfusion, but thereafter diagnosed as NH and reviewed the literature in the view point of the latest developments related to the topic.

  2. Lactobacillus rhamnosus HN001 decreases the severity of necrotizing enterocolitis in neonatal mice and preterm piglets: evidence in mice for a role of TLR9.

    Science.gov (United States)

    Good, Misty; Sodhi, Chhinder P; Ozolek, John A; Buck, Rachael H; Goehring, Karen C; Thomas, Debra L; Vikram, Amit; Bibby, Kyle; Morowitz, Michael J; Firek, Brian; Lu, Peng; Hackam, David J

    2014-06-01

    Necrotizing enterocolitis (NEC) is the leading cause of death from gastrointestinal disease in premature infants and develops partly from an exaggerated intestinal epithelial immune response to indigenous microbes. There has been interest in administering probiotic bacteria to reduce NEC severity, yet concerns exist regarding infection risk. Mechanisms of probiotic activity in NEC are unknown although activation of the microbial DNA receptor Toll-like receptor-9 (TLR9) has been postulated. We now hypothesize that the Gram-positive bacterium Lactobacillus rhamnosus HN001 can attenuate NEC in small and large animal models, that its microbial DNA is sufficient for its protective effects, and that protection requires activation of the Toll-like receptor 9 (TLR9). We now show that oral administration of live or UV-inactivated Lactobacillus rhamnosus HN001 attenuates NEC severity in newborn mice and premature piglets, as manifest by reduced histology score, attenuation of mucosal cytokine response, and improved gross morphology. TLR9 was required for Lactobacillus rhamnosus-mediated protection against NEC in mice, as the selective decrease of TLR9 from the intestinal epithelium reversed its protective effects. Strikingly, DNA of Lactobacillus rhamnosus HN001 reduced the extent of proinflammatory signaling in cultured enterocytes and in samples of resected human ileum ex vivo, suggesting the therapeutic potential of this probiotic in clinical NEC. Taken together, these findings illustrate that Lactobacillus rhamnosus HN001 is an effective probiotic for NEC via activation of the innate immune receptor TLR9 and that Lactobacillus rhamnosus DNA is sufficient for its protective effects, potentially reducing concerns regarding the infectious risk of this novel therapeutic approach. Copyright © 2014 the American Physiological Society.

  3. Comparisons of serum sclerostin levels among patients with postmenopausal osteoporosis, primary hyperparathyroidism and osteomalacia.

    Science.gov (United States)

    Kaji, H; Imanishi, Y; Sugimoto, T; Seino, S

    2011-07-01

    Wnt-β-catenin signaling is important for bone formation. Sclerostin inhibits bone formation mainly by suppressing this signal, and several studies suggest that the suppression of sclerostin expression contributes to the bone anabolic action of parathyroid hormone (PTH). We therefore examined serum sclerostin levels using enzyme-linked immunosolvent assay in 18 patients with postmenopausal osteoporosis, 9 postmenopausal women with primary hyperparathyroidism (pHPT) and 7 patients with osteomalacia. Serum levels of sclerostin were significantly lower in the group with pHPT, compared with those with postmenopausal osteoporosis. Moreover, serum sclerostin levels were significantly lower in the group with tumor-induced osteomalacia, but not in the group with osteomalacia without tumor, compared with those with postmenopausal osteoporosis. In patients with pHPT, serum sclerostin levels were significantly and negatively correlated to serum calcium and PTH levels. In patients with postmenopausal osteoporosis, serum levels of sclerostin levels were significantly and positively related to serum calcium and creatinine levels. In conclusion, we showed that serum sclerostin levels are decreased presumably through endogenous PTH elevation in postmenopausal women with pHPT, compared with the patients with postmenopausal osteoporosis.

  4. THE EFFECT OF CINACALCET (SENSIPAR®) ON INTRAOPERATIVE FINDINGS IN TERTIARY HYPERPARATHYROIDISM PATIENTS UNDERGOING PARATHYROIDECTOMY

    Science.gov (United States)

    Somnay, Yash R.; Weinlander, Eric; Schneider, David F.; Sippel, Rebecca S.; Chen, Herbert

    2014-01-01

    Introduction Tertiary hyperparathyroidism (3HPTH) patients who undergo parathyroidectomy are often managed with calcium lowering medications such as cinacalcet (Sensipar®) preceding surgery. Here, we assess how cinacalcet (Sensipar®) treatment influences intraoperative PTH (IOPTH) kinetics and surgical findings in 3HPTH patients undergoing parathyroidectomy. Methods 116 retrospectively reviewed 3HPTH patients underwent, parathyroidectomy of which 14 were on cinacalcet and 112 were on no drug. IOPTH levels fitted to linear curves vs. time were used to evaluate the role of cinacalcet. Results Cinacalcet did not significantly correlate with rates of cure (p=0.41) or recurrence (p=0.54). Patients on cinacalcet experienced a significantly steeper decline in IOPTH compared to those not on medication (p=0.005). Cinacalcet treatment was associated with a significant increase in rate of hungry bones (p=0.04). Weights of the heaviest glands resected (p=0.02) and preoperative PTH levels (p=0.0004) were significantly higher among patients on cinacalcet. Conclusions Perioperative cinacalcet treatment in 3HPTH patients alters IOPTH kinetics by causing a steeper IOPTH decline, but does not require modifying standard IOPTH protocol. Although cinacalcet use does not adversely affect cure rates, it is associated with higher preoperative PTH and an increased incidence of hungry bones, hence serving as an indicator of more severe disease. Cinacalcet does not need to be held prior to surgery. PMID:25456900

  5. No difference between alfacalcidol and paricalcitol in the treatment of secondary hyperparathyroidism in hemodialysis patients: a randomized crossover trial

    DEFF Research Database (Denmark)

    Hansen, Ditte; Rasmussen, Knud; Danielsen, Henning

    2011-01-01

    Alfacalcidol and paricalcitol are vitamin D analogs used for the treatment of secondary hyperparathyroidism in patients with chronic kidney disease, but have known dose-dependent side effects that cause hypercalcemia and hyperphosphatemia. In this investigator-initiated multicenter randomized...... were equally effective in the suppression of secondary hyperparathyroidism in hemodialysis patients while calcium and phosphorus were kept in the desired range...

  6. A Pleural Solitary Fibrous Tumor, Multiple Gastrointestinal Stromal Tumors, Moyamoya Disease, and Hyperparathyroidism in a Patient Associated with NF1

    Directory of Open Access Journals (Sweden)

    Yoko Yamamoto

    2015-01-01

    Full Text Available Neurofibromatosis type 1 (NF1, also called von Recklinghausen’s disease, is a multisystemic disease caused by an alteration of the NF1 gene, a tumor suppressor located on the long arm of chromosome 17 (17q11.2. Loss of the gene function, due to a point mutation, leads to an increase in cell proliferation and the development of several tumors. We report a 60-year-old female patient manifesting hypercalcemia due to hyperparathyroidism, a solitary fibrous tumor (SFT of the pleura, multiple gastrointestinal stromal tumors (GISTs, and moyamoya disease associated with NF1. The SFT and GISTs were removed by staged operations. Then, hypercalcemia was successfully controlled after resection of the parathyroid adenoma. Based on a literature review, these combinations have never been reported, and the relevant literature is briefly discussed.

  7. Simultaneous Incidental Parathyroid Carcinoma and Intrathyroid Parathyroid Gland in Suspected Renal Failure Induced Hyperparathyroidism.

    Science.gov (United States)

    Pappa, Andrew; Hackman, Trevor

    2017-01-01

    Hyperparathyroidism is a common disorder affecting more than hundreds of thousands of people annually. While most commonly secondary to an adenoma, it may also arise from four-gland hyperplasia or malignancy. In the case of primary hyperparathyroidism, the number of glands involved may be unknown prior to surgery. In contrast, the metabolic disorder associated with renal failure induced hyperparathyroidism ensures a hyperplasia picture. Despite the uniform hyperplasia seen in tertiary disease and the preoperative expectation for four-gland exploration, our case demonstrates the continued need for a surgeon's vigilance during dissection to identify all glands and appropriately use intraoperative parathyroid hormone (PTH) testing. In addition, while intraoperative PTH assessment is an effective method for confirming adequacy of treatment for hyperparathyroidism, only surgical pathology can confirm malignancy, which should be considered with PTH levels > 1,000. The case also underscores the importance of comprehensive surgery management and mindful interpretation of intraoperative PTH levels in the management of hyperparathyroidism. Standard surgical technique includes complete exploration of the central compartment, and thyroid lobectomy when the aforementioned exploration fails to reveal the necessary parathyroid tissue, especially with a persistently elevated PTH. Without a standardized progressive compartment exploration and judicious use of intraoperative hormone testing, intrathyroidal parathyroid glands can be missed.

  8. Radioactive probe-guided parathyroidectomy for secondary hyperparathyroidism

    Institute of Scientific and Technical Information of China (English)

    Yuan Fei; Yu Weijia; Waqas Ahmad; Yang Zhixue; Cao Hua; Li Juncheng; Jiang Guoqin

    2014-01-01

    Background The value of gamma probes in the surgical treatment of secondary hyperparathyroidism (sHPT) was determined.The aim of this study was to enhance the rate of successful total parathyroidectomy in patients with sHPT using intraoperative gamma probe investigations.Methods We retrospectively analyzed the clinical data of 48 sHPT patients between May 2007 and September 2011.Preoperative 99Tcm-methoxyisobutyl isonitrile (MIBI) scintigraphy and high-frequency ultrasonography were used for parathyroid localization.Thirty-five patients (group Ⅰ) underwent conventional neck exploration and open parathyroidectomy.Thirteen patients (group Ⅱ) underwent gamma probe-guided total parathyroidectomy and parathyroid transplantation.The two groups were compared in terms of the number of parathyroid resections,operative time,and postoperative changes in the blood levels of parathyroid hormone (PTH),calcium,and phosphate.Results The clinical manifestations,PTH and calcium levels,age distribution,and clinical characteristics did not differ between the two groups.The accuracy of preoperative 99Tcm-MIBI scintigraphy (89.74%) for the diagnosis of hyperparathyroidism did not differ from that of ultrasonography (81.25%).However,the accuracy of 99TcmMIBI scintigraphy (66.67%) for localizing hyperfunctioning parathyroids was significantly lower than that of ultrasonography (76.86%).The operation time was significantly longer in group Ⅰ ((120±25) minutes) than in group Ⅱ ((90±30) minutes).The accuracy of parathyroid identification was significantly higher in group Ⅱ (92.59%) than in group Ⅰ (80.39%).On average,significantly fewer parathyroid specimens were obtained in group Ⅰ (2.5±0.5) than in group Ⅱ (3.5±0.5).Compared with group Ⅰ,group Ⅱ showed a significant increase (15.4%) in the number of parathyroid resections.The PTH,calcium,and phosphate levels significantly decreased postoperatively in all patients.Conclusions Intraoperative gamma probe

  9. [Hyperparathyroidism secondary to renal insufficiency. Physiopathology, clinicoradiological aspects and treatment].

    Science.gov (United States)

    Ben Hamida, F; Ghazali, A; Boudzernidj, M; Amar, M; Morinière, P; Westeel, P; Fournier, A

    1994-01-01

    Stimulation of PTH secretion and synthesis in chronic renal failure involves direct and indirect factors. The indirect ones are those contributing to a decrease of plasma ionized calcium concentration which stimulates the release of PTH (1) primarily the negative calcium balance due to the iatrogenic reduction of dietary calcium intake associated with an inadequate synthesis of calcitriol, this latter being explained by a reduction in the nephronic mass, the phosphate retention, the acidosis and the retention of uremic toxins (2) more accessorily, the physicochemical dysequilibrium induced by the late occurring hyperphosphatemia. The factors acting directly on the parathyroid gland stimulating synthesis of prepro PTH at its transcription level: not only hypocalcitriolemia but also hypocalcemia and hyperphosphatemia. The clinicoradiological manifestations appear late, mostly only after the patient has been put on dialysis. The most precocious sign is the subperiosteal resorption assessed on the hand X-rays. Therefore diagnosis of hyperparathyroidism relies mainly on the measurement of plasma concentration of intact PTH. In dialysis patients the optimal range corresponding to the best bone histology is between 1 an 3 times the upper limit of normal. No such data exist for predialysis patients. Medical treatment of hyperparathyroidism should primarily be preventive, probably in predialysis lipin patient as soon as plasma intact PTH is greater than the normal upper limit. This treatment is based primarily on the prevention of phosphate retention, of negative calcium balance and acidosis by the use of oral alkaline salts of calcium given with the meals in association with appropriate dietary protein and phosphate restriction. Native vitamin D depletion should also be prevented but use of 1 alpha OH vitamin D3 metabolites in controversial: it is reasonable to administer them only when plasma intent PTH is above 3-7 the normal upper limit and when plasma phosphate is

  10. Neonatal genital prolapse: A case report

    Directory of Open Access Journals (Sweden)

    Sindu Saksono

    2015-04-01

    Full Text Available Neonatal genital prolapse without congenital spinal abnormality is rare. Several treatment modalities are known to manage this condition. Our management consists of manually reducing the prolapsed mass and applying purse string suture technique.

  11. Diagnosis and management of neonatal thrombocytopenia.

    Science.gov (United States)

    Holzhauer, Susanne; Zieger, Barbara

    2011-12-01

    Thrombocytopenia is the most common haematological abnormality in newborns admitted to neonatal care units and serves as an important indicator of underlying pathological processes of mother or child. In most cases thrombocytopenia is mild to moderate and resolves within the first weeks of life without any intervention. However, in some neonates thrombocytopenia is severe or may reflect an inborn platelet disorder. As clinical course and outcome of thrombocytopenia depend on the aetiology of thrombocytopenia, an appropriate work-up is essential to guide therapy in neonates with thrombocytopenia and to avoid severe bleeding.

  12. Knowledge, attitudes and practices of neonatal staff concerning neonatal pain management

    Directory of Open Access Journals (Sweden)

    Sizakele L.T. Khoza

    2014-10-01

    Full Text Available Background: Neonatal pain management has received increasing attention over the past four decades. Research into the effects of neonatal pain emphasises the professional, ethical and moral obligations of staff to manage pain for positive patient outcomes. However, evaluation studies continuously report evidence of inadequate neonate pain management and a gap between theory and practice.Objective: This study reviewed current practice in neonatal pain management to describe the knowledge, attitudes and practices of nurses and doctors regarding pain management for neonates in two academic hospitals.Method: A non-experimental, prospective quantitative survey, the modified Infant Pain Questionnaire, was used to collect data from 150 nurses and doctors working in the neonatal wards of two academic hospitals in central Gauteng.Results: The response rate was 35.33% (n = 53, most respondents being professional nurses (88.68%; n = 47 working in neonatal intensive care units (80.77%; n = 42; 24 (45.28% had less than 5 years’ and 29 respondents 6 or more years’ working experience in neonatal care. A review of pain management in the study setting indicated a preference for pharmacological interventions to relieve moderate to severe pain. An association (p < 0.05 was found between pain ratings on 5 procedures and frequency of administration of pharmacological pain management. Two-thirds of respondents (64% reported that there were no pain management guidelines in the neonatal wards in which they worked.Conclusion: The interventions to manage moderate neonatal pain are in line with international guidelines. However, neonatal pain management may not occur systematically based on prior assessment of neonatal pain, choice of most appropriate intervention and evaluation. This study recommends implementation of a guideline to standardise practice and ensure consistent and adequate pain management in neonates

  13. Mild hyperparathyroidism: a novel surgically correctable feature of primary aldosteronism.

    Science.gov (United States)

    Maniero, Carmela; Fassina, Ambrogio; Seccia, Teresa M; Toniato, Antonio; Iacobone, Maurizio; Plebani, Mario; De Caro, Raffaele; Calò, Lorenzo A; Pessina, Achille C; Rossi, Gian P

    2012-02-01

    The parathyroid hormone (PTH) stimulates aldosterone secretion and cell proliferation in human adrenocortical cells; moreover, in rats hyperaldosteronism was associated with hyperparathyroidism. Hence, PTH could drive aldosterone excess in human primary aldosteronism. To test this hypothesis, we recruited 105 consecutive hypertensive patients, of whom 44 had primary aldosteronism due to an aldosterone-producing adenoma (APA) and 61 had primary (essential) hypertension. We measured the plasma levels of (1-84)-PTH, 25(OH)D, 1,25(OH)2D, and serum Ca (total and ionized), inorganic P, Mg, K, and the 24-h urinary excretion of Ca, P, and deoxypyridinoline. In primary aldosteronism patients, these measurements were repeated after adrenalectomy or mineralocorticoid receptor blockade. We also sought for PTH receptor (PTHR-1) mRNA and protein in APA tissue. Compared with primary (essential) hypertension patients, those with primary aldosteronism showed significantly higher plasma PTH (+31%), despite comparable urinary Ca excretion and similarly deficient 25(OH) vitamin D levels. In APA patients, who showed the PTHR-1 transcript and protein in tumor tissue, adrenalectomy normalized PTH levels (from 118 ± 13 to 76 ± 12 ng/l; P = 0.002) and increased ionized Ca(from 1.17 ± 0.04 to 1.22 ± 0.03 mmol/l; P PTHR-1 in APA might contribute to maintaining hyperaldosteronism despite suppression of angiotensin II formation.

  14. Secondary hyperparathyroidism to chronic renal disease in dialysis patients in Para– Brazil

    Directory of Open Access Journals (Sweden)

    Georgia Miranda Tomich

    2015-12-01

    Full Text Available Objective: to establish the frequency of secondary hyperparathyroidism on renal replacement therapy patients on the nephrology service of southeast Para (Brazil.Methods: retrospective cross-sectional survey based on available electronic medical records data referring to the first semester of 2014.Results: data from 108 patients with an average age of 47.8 ± 12.0 years (20-65 were analyzed, 64 patients (59.3% were male. The frequency of secondary hyperparathyroidism was 57.4%, corresponding to a total of 62 patients with parathyroid hormone above 300 pg/ml. Parathyroid hormone levels greater than 1000 pg/ml were found in 12.0% (n=13 of the sample.Conclusion: the occurrence of secondary hyperparathyroidism was similar to other retrospective studies published. This data collection can contribute to improve the assistance program for dialysis patients.

  15. Brown tumor mimicking maxillary sinus mucocele as the first manifestation of primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Guldfred, Liviu-Adelin; Daugaard, Søren; von Buchwald, Christian

    2012-01-01

    We describe the first case of brown tumor mimicking a maxillary sinus mucocele as the first manifestation of the patient's primary hyperparathyroidism. A 34-year old woman presented with a 14 days history of elevation of the right orbit, retrobulbar pain and cheek anesthesia. The CT and MR...... evaluation showed a mass, initially described as mucocele of the right maxillary sinus. The laboratory studies revealed hyperparathyroidism. The patient underwent acute surgery, and the mass appeared clinically as mucocele. The histological examination of the resected lesion revealed changes representing...... either giant cell granuloma or brown tumor. The finding of hyperparathyroidism confirmed the diagnosis of brown tumor. To our knowledge, this is the first report of cystic brown tumor mimicking a mucocele of the maxillary sinus....

  16. Treatment of secondary hyperparathyroidism in haemodialysis patients: a randomised clinical trial comparing paricalcitol and alfacalcidol

    DEFF Research Database (Denmark)

    Hansen, Ditte; Brandi, Lisbet; Rasmussen, Knud

    2009-01-01

    BACKGROUND: Secondary hyperparathyroidism is a common feature in patients with chronic kidney disease. Its serious clinical consequences include renal osteodystrophy, calcific uremic arteriolopathy, and vascular calcifications that increase morbidity and mortality.Reduced synthesis of active...... vitamin D contributes to secondary hyperparathyroidism. Therefore, this condition is managed with activated vitamin D. However, hypercalcemia and hyperphosphatemia limit the use of activated vitamin D.In Denmark alfacalcidol is the primary choice of vitamin D analog.A new vitamin D analog, paricalcitol...... hyperparathyroidism and the tendency towards hyperphosphatemia and hypercalcemia. METHODS/DESIGN: This is an investigator-initiated cross-over study. Nine Danish haemodialysis units will recruit 117 patients with end stage renal failure on maintenance haemodialysis therapy.Patients are randomised into two treatment...

  17. SECONDARY HYPERPARATHYROIDISM AFTER BARIATRIC SURGERY: TREATMENT IS WITH CALCIUM CARBONATE OR CALCIUM CITRATE?

    Science.gov (United States)

    BARETTA, Giorgio Alfredo Pedroso; CAMBI, Maria Paula Carlini; RODRIGUES, Arieli Luz; MENDES, Silvana Aparecida

    2015-01-01

    Background : Bariatric surgery, especially Roux-en-Y gastric bypass, can cause serious nutritional complications arising from poor absorption of essential nutrients. Secondary hyperparathyroidism is one such complications that leads to increased parathyroid hormone levels due to a decrease in calcium and vitamin D, which may compromise bone health. Aim : To compare calcium carbonate and calcium citrate in the treatment of secondary hyperparathyroidism. Method : Patients were selected on the basis of their abnormal biochemical test and treatment was randomly done with citrate or calcium carbonate. Results : After 60 days of supplementation, biochemical tests were repeated, showing improvement in both groups. Conclusion : Supplementation with calcium (citrate or carbonate) and vitamin D is recommended after surgery for prevention of secondary hyperparathyroidism. PMID:26537273

  18. An analysis of preoperative localization of parathyroid glands in hyperparathyroidism associated with thyroid diseases

    Energy Technology Data Exchange (ETDEWEB)

    Komatsu, Makoto; Inoue, Kazuaki [Fujimi-kogen Hospital, Nagano (Japan); Itoh, Atsuko

    1996-05-01

    Recently hyperparathyroidism associated with some thyroid diseases, especially nonmedually thyroid carcinoma has been payed attention to. In this study we analyzed 12 cases of hyperparathyroidism (6 cases independent of thyroid diseases and 6 cases associated with thyroid diseases) and estimated the affect of association with thyroid diseases on the preoperative localization of the parathyroid glands. The results of preoperative localization of the parathyroid glands in cases independent of thyroid diseases were relatively satisfactory. On the other hand, the preoperative localization in cases associated with thyroid diseases came to false result in about half of them. It was far from satisfactory. Association of thyroid diseases strongly affected the preoperative localization of the parathyroid glands in hyperparathyroidism. Conventional imaging such as ultrasonography, CT, MRI and {sup 201}Tl-{sup 99m}TC subtraction scintigraphy alone were not satisfactory. Now {sup 99m}Tc-MIBI scintigraphy is expected to be one of reliable imaging methods for progress in the preoperative localization. (author)

  19. Peptic Ulcer Perforation as the First Manifestation of Previously Unknown Primary Hyperparathyroidism

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    Eleni I. Efremidou

    2007-06-01

    Full Text Available A patient admitted for acute abdomen was incidentally found with elevated serum calcium level. In surgery, under conservative treatment of the hypercalcemia, a perforated duodenal ulcer was found and simple closure was performed. Postoperatively, calcium level continued to rise, parathyroid hormone was elevated and ultrasonographic examination showed a lesion in the right anterior neck, while serum gastrin level was normal, thus documenting the diagnosis of primary hyperparathyroidism. Conservative treatment had no effect on calcium level and the patient was subjected to emergency neck exploration, where a large parathyroid adenoma was removed. After surgery, calcium and PTH levels were normalized and the patient was discharged on the 5th postoperative day. Peptic ulcer and its complications are usual manifestations of primary hyperparathyroidism, with or without increased gastrin level. On the other hand, cases of a perforation of peptic ulcer as the first clinical manifestation of primary hyperparathyroidism are extremely rare.

  20. Brown tumor of the maxillary sinus in a patient with primary hyperparathyroidism: a case report

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    Proimos Efklidis

    2009-07-01

    Full Text Available Abstract Introduction Brown tumors are rare focal giant-cell lesions that arise as a direct result of the effect of parathyroid hormone on bone tissue in some patients with hyperparathyroidism. Brown tumors can affect the mandible, maxilla, clavicle, ribs, and pelvic bones. Therefore, diagnosis requires a systemic investigation for lesion differentiation. Case presentation We present a 42-year-old Greek woman, with a rare case of brown tumor of the maxillary sinus due to primary hyperparathyroidism. Primary hyperparathyroidism is caused by a solitary adenoma in 80% of cases and by glandular hyperplasia in 20%. Conclusions Differential diagnosis is important for the right treatment choice. It should exclude other giant cell lesions that affect the maxillae.

  1. Let-7 and MicroRNA-148 Regulate Parathyroid Hormone Levels in Secondary Hyperparathyroidism.

    Science.gov (United States)

    Shilo, Vitali; Mor-Yosef Levi, Irit; Abel, Roy; Mihailović, Aleksandra; Wasserman, Gilad; Naveh-Many, Tally; Ben-Dov, Iddo Z

    2017-03-15

    Secondary hyperparathyroidism commonly complicates CKD and associates with morbidity and mortality. We profiled microRNA (miRNA) in parathyroid glands from experimental hyperparathyroidism models and patients receiving dialysis and studied the function of specific miRNAs. miRNA deep-sequencing showed that human and rodent parathyroids share similar profiles. Parathyroids from uremic and normal rats segregated on the basis of their miRNA expression profiles, and a similar finding was observed in humans. We identified parathyroid miRNAs that were dysregulated in experimental hyperparathyroidism, including miR-29, miR-21, miR-148, miR-30, and miR-141 (upregulated); and miR-10, miR-125, and miR-25 (downregulated). Inhibition of the abundant let-7 family increased parathyroid hormone (PTH) secretion in normal and uremic rats, as well as in mouse parathyroid organ cultures. Conversely, inhibition of the upregulated miR-148 family prevented the increase in serum PTH level in uremic rats and decreased levels of secreted PTH in parathyroid cultures. The evolutionary conservation of abundant miRNAs in normal parathyroid glands and the regulation of these miRNAs in secondary hyperparathyroidism indicates their importance for parathyroid function and the development of hyperparathyroidism. Specifically, let-7 and miR-148 antagonism modified PTH secretion in vivo and in vitro, implying roles for these specific miRNAs. These findings may be utilized for therapeutic interventions aimed at altering PTH expression in diseases such as osteoporosis and secondary hyperparathyroidism.

  2. Biochemical and Clinical Variables of Normal Parathyroid and Hyperparathyroid Diabetic Chronic Kidney Disease Patients

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    Syed Abdul Kader

    2016-09-01

    Full Text Available Background: In chronic kidney disease (CKD intact parathyroid hormone (iPTH level is often increased before clinical hyperphosphatemia occurs. Despite its importance very few studies evaluated parathyroid status in CKD. Objective: The study was undertaken to estimate level of parathormone in diabetic CKD patients at a tertiary level hospital and assessing its relationship with different parameters like hemoglobin, calcium etc. and comparing biochemical and clinical variables between normal parathyroid and hyperparathyroid groups. Materials and Methods: It was a hospital based cross-sectional study involving purposively selected chronic kidney disease patients attending nephrology and endocrinology outdoor and indoor services of BIRDEM hospital, Dhaka, Bangladesh. Study was conducted during the period of April to October 2010. All the subjects were divided into two groups based on serum parathormone level and different parameters were compared between groups. Results: The mean duration of chronic kidney disease was significantly higher in hyperparathyroid group than that in the normal group (<0.001. Retinopathy and hypertension were more common in hyperparathyroid group than that in patients with normal serum parathormone (p<0.001 and p=0.012. Neuropathy was solely present in hyperparathyroid group (p<0.001. Mean fasting blood glucose, serum creatinine and serum phosphate were significantly higher in the hyperparathyroid group compared to normal group (p<0.001 in all cases while the mean serum calcium and haemoglobin were lower in hyperparathyroid group than those in the normal group (p<0.001 in both cases. Serum creatinine and serum parathormone bears a significantly linear relationship (r=0.986, p<0.001, while serum parathormone and serum calcium bears a significantly negative relationship (r=−0.892 and p<0.001. Conclusion: Earlier intervention on the basis of iPTH in addition to other biochemical parameters of chronic kidney disease is

  3. Ethical issues in neonatal intensive care

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    Marcello M. Orzalesi

    2011-01-01

    Full Text Available Recent progress in neonatal care have significantly improved the prognosis and chances of survival of critically ill or extremely preterm neonates and have modified the limits of viability. However, in some circumstances, when the child's death can only be briefly postponed at the price of severe suffering, or when survival is associated with severe disabilities and an intolerable life for the child and his/her parents, the application of the full armamentarium of modern neonatal intensive care may not be appropriate. In such circumstances the limitation of intensive treatments (withholding or withdrawing and shift towards palliative care, can represent a more humane and reasonable alternative. This article examines and discusses the ethical principles underlying such difficult decisions, the most frequent situations in which these decisions may be considered, the role of parents in the decisional process, and the opinions and behaviours of neonatologists of several European neonatal intensive units as reported by the EURONIC study.

  4. Ethical issues in neonatal intensive care.

    Science.gov (United States)

    Orzalesi, Marcello M; Cuttini, Marina

    2011-01-01

    Recent progress in neonatal care have significantly improved the prognosis and chances of survival of critically ill or extremely preterm neonates and have modified the limits of viability. However, in some circumstances, when the child's death can only be briefly postponed at the price of severe suffering, or when survival is associated with severe disabilities and an intolerable life for the child and his/her parents, the application of the full armamentarium of modern neonatal intensive care may not be appropriate. In such circumstances the limitation of intensive treatments (withholding or withdrawing) and shift towards palliative care, can represent a more humane and reasonable alternative. This article examines and discusses the ethical principles underlying such difficult decisions, the most frequent situations in which these decisions may be considered, the role of parents in the decisional process, and the opinions and behaviours of neonatologists of several European neonatal intensive units as reported by the EURONIC study.

  5. OXYTOCIN INDUCED NEONATAL HYPERBILIRUBINEMIA

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    Smita S.

    2015-05-01

    Full Text Available INTRODUCTION: Hyperbilirubinemia is one of the most common causes of health problems, observed in 60% of term and 80% of preterm infants in the first week of life . Hyperbilirubinemia leads to neurotoxicity in severe condition. Some studies suggests that liberal use of oxytocin for inducing labour is one of the factor which lead to neonatal hyperbilirubinemia. OBJECTIVE: To compare the effect of oxytocin and neonatal bilirubin levels with spontaneous vaginal delivery . MATERIALS AND METHOD S : 100 full term parturients were selected for this study. The subjects were divided into two groups. 50 healthy babies of women who had oxytocin induced labour and 50 healthy babies of women with normal vaginal delivery following spontaneous onset of labour formed the control group. Neon atal serum bilirubin was measured on day 1, 3 and 5 after delivery. Bilirubin was measured by spectrophotometry. Data was analysed in ms excel sheet using spss 19.0v. Statistical analysis was done by using unpaired‘t’ test. RESULTS: There was significant i ncrease in bilirubin level in oxytocin induced group compared to control group on day 1 and 3. There was insignificant increase in bilirubin level in oxytocin induced group on day 5. However the level of serum bilirubin is within normal limits as bilirubin level normally rises on till 4 th day and decreases thereafter. CONCLUSION: Neonatal hyperbilirubinemia may be due to oxytocin administration by continues IV infusion which results in erythrocyte swell and rupture. Increase in bilirubin level in oxytocin i nduced group is within physiological limits

  6. Effect of surgery on cardiac structure and function in mild primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Persson, Anita; Bollerslev, Jens; Rosen, Thord

    2011-01-01

    The cardiovascular (CV) risk profile is worsened in primary hyperparathyroidism (PHPT), and CV mortality is related to serum calcium levels. It is unknown whether CV mortality is increased in the most common form of PHPT and whether the increased CV risk is reversible after surgery.......The cardiovascular (CV) risk profile is worsened in primary hyperparathyroidism (PHPT), and CV mortality is related to serum calcium levels. It is unknown whether CV mortality is increased in the most common form of PHPT and whether the increased CV risk is reversible after surgery....

  7. Hyperparathyroidism Two Years after Radioactive Iodine Therapy in an Adolescent Male

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    Danielle L. Gomez

    2014-01-01

    Full Text Available Primary hyperparathyroidism is a very rare complication following radioactive iodine therapy. There is typically a latency period of more than a decade following radiation exposure and, therefore, it is observed almost exclusively in adults. Consequently, pediatricians are not aware of the association. We present a case of primary hyperparathyroidism due to a solitary parathyroid adenoma occurring in an adolescent male two years following radioactive iodine treatment for papillary thyroid carcinoma. Periodic screening of serum calcium following ablative doses of radioactive iodine for thyroid cancer may be justified even in adolescents.

  8. Self-diagnosis of hyperparathyroidism during pregnancy resulting in parathyroidectomy and uncomplicated delivery.

    Science.gov (United States)

    Medza, Aleksandra; Obolonczyk, Lukasz; Lewalska, Anna; Buss, Tomasz; Peksa, Rafal; Siekierska-Hellmann, Malgorzata; Berendt-Obolonczyk, Monika; Wisniewski, Piotr; Sworczak, Krzysztof

    2017-03-06

    Primary hyperparathyroidism is a condition with hypercalcemia and elevated parathyroid hormone (PTH). Typically, treating patients with such disease does not pose a problem for doctors, unless the patient is pregnant. Firstly, pregnancy may mask signs of hypercalcemia. Secondly, treatment should be applied with special care for immature fetus. If undiagnosed and untreated, it is life-threatening for the mother and the baby. The main cause of primary hyperparathyroidism is parathyroid adenoma, which should be removed surgically in second trimester. If the patient is monitored by a multidisciplinary team, the risk of mortality and pregnancy loss is reduced.

  9. Increased mortality in patients hospitalized with primary hyperparathyroidism during the period 1977-1993 in Denmark

    DEFF Research Database (Denmark)

    Øgard, Christina G; Engholm, Gerda; Almdal, Thomas P

    2004-01-01

    The aim of the present study was to determine whether patients with the incident hospital diagnosis of primary hyperparathyroidism (PHPT) in Denmark during the period 1977-1993 had an increased mortality from cardiovascular disease and cancer compared to the rest of the Danish population. In a ra......The aim of the present study was to determine whether patients with the incident hospital diagnosis of primary hyperparathyroidism (PHPT) in Denmark during the period 1977-1993 had an increased mortality from cardiovascular disease and cancer compared to the rest of the Danish population...

  10. Brown tumor: clinical findings of secondary hyperparathyroidism in patients with renal osteodystrophy.

    Science.gov (United States)

    Silva, Mairaira Teles Leão E; Cedraz, Juliana Silva Barros; Pontes, Caetano Guilherme Carvalho; Trento, Cleverson Luciano; Brasileiro, Bernardo Ferreira; Piva, Marta Rabello; Pereira, Fabiano Alvim

    2017-01-01

    A brown tumor, or osteoclastoma, is a nonneoplastic bony lesion associated with hyperparathyroidism and directly related to increased levels of parathyroid hormone. These tumors result from excessive osteoclastic activity. This article presents 3 cases of brown tumor localized in facial bones. The lesions were the result of secondary hyperparathyroidism associated with chronic renal failure. The patients were two 42-year-old men and a 39-year-old woman. All patients had been treated systemically by hemodialysis for more than 10 years. This article highlights the importance of proper diagnosis and management of dental patients presenting with a brown tumor.

  11. Diuretics, calciuria and secondary hyperparathyroidism in the Chronic Renal Insufficiency Cohort

    Science.gov (United States)

    Isakova, Tamara; Anderson, Cheryl A. M.; Leonard, Mary B.; Xie, Dawei; Gutiérrez, Orlando M.; Rosen, Leigh K.; Theurer, Jacquie; Bellovich, Keith; Steigerwalt, Susan P.; Tang, Ignatius; Anderson, Amanda Hyre; Townsend, Raymond R.; He, Jiang; Feldman, Harold I.; Wolf, Myles

    2011-01-01

    Background. Secondary hyperparathyroidism is a common complication of chronic kidney disease (CKD) that is associated with bone disease, cardiovascular disease and death. Pathophysiological factors that maintain secondary hyperparathyroidism in advanced CKD are well-known, but early mechanisms of the disease that can be targeted for its primary prevention are poorly understood. Diuretics are widely used to control volume status and blood pressure in CKD patients but are also known to have important effects on renal calcium handling, which we hypothesized could alter the risk of secondary hyperparathyroidism. Methods. We examined the relationship of diuretic treatment with urinary calcium excretion, parathyroid hormone (PTH) levels and prevalence of secondary hyperparathyroidism (PTH ≥ 65 pg/mL) in a cross-sectional study of 3616 CKD patients in the Chronic Renal Insufficiency Cohort. Results. Compared with no diuretics, treatment with loop diuretics was independently associated with higher adjusted urinary calcium (55.0 versus 39.6 mg/day; P < 0.001), higher adjusted PTH [67.9, 95% confidence interval (CI) 65.2–70.7 pg/mL, versus 52.8, 95% CI 51.1–54.6 pg/mL, P < 0.001] and greater odds of secondary hyperparathyroidism (odds ratio 2.1; 95% CI 1.7–2.6). Thiazide monotherapy was associated with lower calciuria (25.5 versus 39.6 mg/day; P < 0.001) but only modestly lower PTH levels (50.0, 95% CI 47.8–52.3, versus 520.8, 95% CI 51.1–54.6 pg/mL, P = 0.04) compared with no diuretics. However, coadministration of thiazide and loop diuretics was associated with blunted urinary calcium (30.3 versus 55.0 mg/day; P <0.001) and odds of hyperparathyroidism (odds ratio 1.3 versus 2.1; P for interaction = 0.05) compared with loop diuretics alone. Conclusions. Loop diuretic use was associated with greater calciuria, PTH levels and odds of secondary hyperparathyroidism compared to no treatment. These associations were attenuated in patients who were coadministered

  12. An LRP5 receptor with internal deletion in hyperparathyroid tumors with implications for deregulated WNT/beta-catenin signaling.

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    Peyman Björklund

    2007-11-01

    Full Text Available BACKGROUND: Hyperparathyroidism (HPT is a common endocrine disorder with incompletely understood etiology, characterized by enlarged hyperactive parathyroid glands and increased serum concentrations of parathyroid hormone and ionized calcium. We have recently reported activation of the Wnt signaling pathway by accumulation of beta-catenin in all analyzed parathyroid tumors from patients with primary HPT (pHPT and in hyperplastic parathyroid glands from patients with uremia secondary to HPT (sHPT. Mechanisms that may account for this activation have not been identified, except for a few cases of beta-catenin (CTNNB1 stabilizing mutation in pHPT tumors. METHODS AND FINDINGS: Reverse transcription PCR and Western blot analysis showed expression of an aberrantly spliced internally truncated WNT coreceptor low-density lipoprotein receptor-related protein 5 (LRP5 in 32 out of 37 pHPT tumors (86% and 20 out of 20 sHPT tumors (100%. Stabilizing mutation of CTNNB1 and expression of the internally truncated LRP5 receptor was mutually exclusive. Expression of the truncated LRP5 receptor was required to maintain the nonphosphorylated active beta-catenin level, transcription activity of beta-catenin, MYC expression, parathyroid cell growth in vitro, and parathyroid tumor growth in a xenograft severe combined immunodeficiency (SCID mouse model. WNT3 ligand and the internally truncated LRP5 receptor strongly activated transcription, and the internally truncated LRP5 receptor was insensitive to inhibition by DKK1. CONCLUSIONS: The internally truncated LRP5 receptor is strongly implicated in deregulated activation of the WNT/beta-catenin signaling pathway in hyperparathyroid tumors, and presents a potential target for therapeutic intervention.

  13. Percutaneous ethanol injection therapy for advanced renal hyperparathyroidism in Japan: 2004 survey by the Japanese Society for Parathyroid Intervention.

    Science.gov (United States)

    Kakuta, Takatoshi; Fukagawa, Masafumi; Kitaoka, Masafumi; Koiwa, Fumihiko; Onoda, Noritaka; Tominaga, Yasuhiro; Akizawa, Tadao; Kurokawa, Kiyoshi

    2008-08-01

    Background. Marked hyperplasia of the parathyroid gland (PTG) is a characteristic feature of severe hyperparathyroidism in patients under chronic haemodialysis treatment. Percutaneous ethanol injection therapy (PEIT) is now becoming popular in Japan as a treatment option for secondary hyperparathyroidism (SHPT) and its cost is covered by the National Health Insurance (NHI) System. The Japanese Society for Parathyroid Intervention surveyed its membership in 2004 to revise the guidelines for the use of PEIT. Methods. The project was approved by the Executive Committee of the Society, and the primary questionnaire was addressed to 3268 centres (departments) affiliated with the Japanese Society for Dialysis Therapy. A follow-up questionnaire was sent to all the centres that responded. Results. Although the number of centres to which the questionnaire was sent in 2004 was 3268, compared with 2653 in 1998, the number of responses decreased from 1425 (53.7%) in 1998 to 962 (29.4%) in 2004. To the question of whether the centre performed PEIT, 114 (11.9%) answered 'Yes' and 848 (88.1%) answered 'No' in 2004. It was an increase from 1998 when only 83 (5.8%) of 1425 centres answered 'Yes'. In the 1998 survey, 612 patients underwent PEIT at 74 centres, and in 2004, 2098 patients underwent PEIT at 111 centres. Conclusions. PEIT may become the frequently performed treatment for SHPT patients who become resistant to medical therapy. However, the same problems as in 1998 remain unsolved; that is, recurrent nerve paralysis, difficulty of post-PEIT PTx and lack of evidence showing the long-term effectiveness of PEIT.

  14. Routine neonatal circumcision?

    OpenAIRE

    Tran, P. T.; Giacomantonio, M.

    1996-01-01

    Routine neonatal circumcision is still a controversial procedure. This article attempts to clarify some of the advantages and disadvantages of neonatal circumcision. The increased rate of penile cancer among uncircumcised men appears to justify the procedure, but that alone is not sufficient justification. The final decision on neonatal circumcision should be made by parents with balanced counsel from attending physicians.

  15. Drug binding properties of neonatal albumin

    DEFF Research Database (Denmark)

    Brodersen, R; Honoré, B

    1989-01-01

    Neonatal and adult albumin was isolated by gel chromatography on Sephacryl S-300, from adult and umbilical cord serum, respectively. Binding of monoacetyl-diamino-diphenyl sulfone, warfarin, sulfamethizole, and diazepam was studied by means of equilibrium dialysis and the binding data were analyzed...... by the method of several acceptable fitted curves. It was found that the binding affinity to neonatal albumin is less than to adult albumin for monoacetyl-diamino-diphenyl sulfone and warfarin. Sulfamethizole binding to the neonatal protein is similarly reduced when more than one molecule of the drug is bound...

  16. Neonatal uterine prolapse - a case report.

    Science.gov (United States)

    Saha, D K; Hasan, K M; Rahman, S M; Majumder, S K; Zahid, M K; Chakraborty, A K; Bari, M S

    2014-04-01

    Uterine prolapse is commonly seen in the geriatric age group. Congenital vaginouterine prolapse is a rare condition occurring in neonates and is usually associated with spinal cord malformations in about 85% of cases. Several modalities of treatment have been described for neonatal uterine prolapse. Conservative treatment in the form of simple digital reposition, use of pessary or other self-retaining device is usually sufficient to treat this condition, which is self-limiting and regressive. Here we report our first case of neonatal uterine prolapse, managed successfully with simple digital reposition.

  17. CINACALCET IN TREATMENT OF HYPERPARATHYROIDISM IN RECIPIENTS OF RENAL GRAFT

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    O. N. Vetchinnikova

    2014-01-01

    Full Text Available Aim. Evaluate the efficacy and safety of cinacalcet in the treatment of hyperparathyroidism (HPT in renal transplant recipients. Materials and methods. During the year, three patients with satisfactory functioning kid- ney transplant (glomerular filtration rate − GFR 44–80 ml/min and HPT (parathyroid hormone − PTH 320– 348 pg/ml, resistant to treatment with active forms of vitamin D and hypercalcemia (2,6–3,1 mmol/l were treated with cinacalcet (initial dose of 30 mg/day, supporting − 60–15 mg/day with the added in 2–3 months alfacalcidol (0,25–0,75 μg/day. Investigated the serum concentrations and renal excretion of calcium and phos- phorus, PTH, renal transplant function (blood creatinine, GFR, plasma concentrations of tacrolimus, bone mine- ral density (BMD in different parts of the skeleton (dual energy X-ray absorptiometry. Results. A month later, the level of calcium in the blood to normal, PTH levels decreased by 1,2–3,2 times. A year later, in two patients, blood levels of PTH was back to normal, one − up − 142 pg/ml. Renal excretion of calcium varied differently − in two patients increased gradually, without exceeding the physiological norm, and in one − remained stable. Gene- ral pattern in the dynamics of serum concentration and urinary excretion of phosphorus was not observed. Renal graft function remained stable − GFR 46–76 ml/min. BMD of the distal forearm, femoral neck and lumbar spine in two patients remained the same, in one − increased by 14, 6 and 7%. Adverse events were absent. Conclusion. Application of cinacalcet is promising for the correction of HPT in renal transplant recipients. 

  18. Vitamin D status in primary hyperparathyroidism: effect of genetic background.

    Science.gov (United States)

    Battista, Claudia; Guarnieri, Vito; Carnevale, Vincenzo; Baorda, Filomena; Pileri, Mauro; Garrubba, Maria; Salcuni, Antonio S; Chiodini, Iacopo; Minisola, Salvatore; Romagnoli, Elisabetta; Eller-Vainicher, Cristina; Santini, Stefano A; Parisi, Salvatore; Frusciante, Vincenzo; Fontana, Andrea; Copetti, Massimiliano; Hendy, Geoffrey N; Scillitani, Alfredo; Cole, David E C

    2017-01-01

    Primary hyperparathyroidism (PHPT) is associated with hypovitaminosis D as assessed by serum total 25-hydroxyvitamin D (TotalD) levels. The aim of this study is to evaluate whether this is also the case for the calculated bioavailable 25-hydroxyvitamin D (BioD) or free 25-hydroxyvitamin D (FreeD), and whether the vitamin D status is influenced by genetic background. We compared vitamin D status of 88 PHPT patients each with a matched healthy family member sharing genetic background, i.e., first-degree relative (FDR), or not, namely an in-law relative (ILR). We compared TotalD and vitamin D-binding protein (DBP), using the latter to calculate BioD and FreeD. We also genotyped two common DBP polymorphisms (rs7041 and rs4588) likely to affect the affinity for and levels of vitamin D metabolites. TotalD was lower (p < 0.001) in PHPT (12.3 ± 6.6 ng/mL) than either family member group (FDR: 19.4 ± 12.1 and ILR: 23.2 ± 14.1), whether adjusted for DBP or not. DBP levels were also significantly lower (p < 0.001) in PHPT (323 ± 73 mg/L) versus FDR (377 ± 98) or ILR (382 ± 101). The differences between PHPT and control groups for TotalD, BioD, and FreeD were maintained after adjustment for season, gender, and serum creatinine. 25-hydroxyvitamin D, evaluated as total, free, or bioavailable fractions, is decreased in PHPT. No difference was seen between first-degree relative and in-law controls, suggesting that neither genetic nor non-genetic background greatly influences the genesis of the hypovitaminosis D seen in PHPT.

  19. Regional cerebral blood flow abnormalities in patients with primary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Cermik, Tevfik F. [Hospital of the University of Trakya, Department of Nuclear Medicine, Edirne (Turkey); Trakya Universitesi Hastanesi, Nukleer Tip Anabilim Dali, Gullapoglu Yerleskesi, Edirne (Turkey); Kaya, Meryem; Bedel, Deniz; Berkarda, Sakir; Yigitbasi, Oemer N. [Hospital of the University of Trakya, Department of Nuclear Medicine, Edirne (Turkey); Ugur-Altun, Betuel [Hospital of the University of Trakya, Department of Internal Medicine, Division of Endocrinology, Edirne (Turkey)

    2007-04-15

    We assessed the alterations in regional cerebral blood flow (rCBF) in patients with primary hyperparathyroidism (PHP) before parathyroidectomy by semiquantitative analysis of brain single photon emission computed tomography (SPECT) images. Included in this prospective study were 14 patients (mean age 47.6 {+-} 10.4 years; 3 male, 11 female) and 10 control subjects (mean age 36.0 {+-} 8.5 years, 6 male, 4 female) were SPECT imaging was performed using a dual-headed gamma camera 60-90 min after intravenous administration of 925 MBq Tc-99m HMPAO. The corticocerebellar rCBF ratios were calculated from 52 brain areas and reference lower values (RLVs) were calculated from the rCBF ratios of control subjects. The regional ratios that were below the corresponding RLV were considered abnormal (hypoperfused). Hypoperfusion was shown in 171 out of 728 regions (23%) and there was a significant correlation between serum calcium, PTH levels and the sum of hypoperfused regions in the patient group (R = 0.75 and P = 0.001, and R = 0.75, P = 0.001, respectively). Significantly reduced rCBF were found in the following cortical regions: bilateral cingulate cortex, superior and inferior frontal cortex, anterior temporal cortex, precentral gyrus, postcentral gyrus and parietal cortex, and right posterior temporal cortex. Our results indicate that alterations in rCBF in patients with PHP can be demonstrated with brain SPECT. The correlation between serum calcium, PTH levels and the sum of hypoperfused regions indicates that there may be a strong relationship between rCBF abnormalities and increased levels of serum calcium and PTH. In addition, the degree of rCBF abnormalities could be determined by brain SPECT in PHP patients with or without psychiatric symptoms. (orig.)

  20. Calcitriol resistance in hemodialysis patients with secondary hyperparathyroidism.

    Science.gov (United States)

    Negri, Armando L; Brandenburg, Vincent M; Brandemburg, Vincent M

    2014-06-01

    Nonselective vitamin D receptor activators (VDRA), such as calcitriol and alfacalcidol, have been successfully used in the treatment of secondary hyperparathyroidism (SHPT) in hemodialysis. Despite their beneficial effects on the control of serum PTH levels, their use has been limited by intolerance (development of hypercalcemia and hyperphosphatemia with consecutive cardiovascular toxicity). Apart from becoming intolerant, in 20-30 % of patients who use nonselective VDRA, serum PTH levels do not decrease appropriately despite increasing doses of these agents. These patients are considered calcitriol-resistant patients. Thus, calcitriol resistance and intolerance are two sides of the same coin: active vitamin D failure. Despite the clinical relevance of active vitamin D failure, definitions of resistance and intolerance are imprecise and have varied over time. More selective VDRA claim to produce less hypercalcemia and hyperphosphatemia and could help clinicians to overcome intolerance. Also, some studies have also shown that paricalcitol can be even useful in resistant patients. Significant limitations of iPTH as a reliable and useful clinical biomarker have been increasingly appreciated. There is evidence that intact PTH concentration must differ by 72 % between any two measurements before it can be considered a significant change. VDR polymorphisms could be involved in the development of SHPT in CKD patients. Interestingly, a higher incidence of the b allele of the VDR BsmI gene variant has been shown to be present in SHPT. The BsmI genotype can also affect the response of hemodialysis to IV calcitriol. A challenge for the future will be to establish biomarkers such as laboratory determinations or ultrasound findings that can help us to early identify those patients who will not respond appropriately to calcitriol or exhibit intolerable side effects .

  1. Pharmacogenetic analysis of cinacalcet response in secondary hyperparathyroidism patients

    Directory of Open Access Journals (Sweden)

    Jeong S

    2016-07-01

    Full Text Available Sohyun Jeong,1 In-Wha Kim,1 Kook-Hwan Oh,2 Nayoung Han,1 Kwon Wook Joo,2 Hyo Jin Kim,2 Jung Mi Oh1 1College of Pharmacy, Research Institute of Pharmaceutical Sciences, Seoul National University, 2Department of Internal Medicine, Division of Nephrology, Seoul National University Hospital, Seoul, Korea Background: Secondary hyperparathyroidism (SHPT is one of the major risk factors of morbidity and mortality in end-stage renal disease. Cinacalcet effectively controls SHPT without causing hypercalcemia and hyperphosphatemia. However, there is significant inter-individual response variance to cinacalcet treatment. Therefore, we aimed to evaluate the genetic effects related with parathyroid hormone regulation as factors for cinacalcet response variance. Methods: Patients with a diagnosis of SHPT based on intact parathyroid hormone (iPTH >300 pg/mL on dialysis were included in this study. They were over 18 years and have been treated by cinacalcet for more than 3 months. Responders and nonresponders were grouped by the serum iPTH changes. Twenty-four single nucleotide polymorphisms of CASR, VDR, FGFR1, KL, ALPL, RGS14, NR4A2, and PTHLH genes were selected for the pharmacogenetic analysis. Results: After adjusting for age, sex, and calcium level, CASR rs1042636 (odds ratio [OR]: 0.066, P=0.027 and rs1802757 (OR: 10.532, P=0.042 were associated with cinacalcet response. The association of haplotypes of CASR rs1042636, rs10190, and rs1802757; GCC (OR: 0.355, P=0.015; and ATT (OR: 2.769, P=0.014 with cinacalcet response was also significant. Conclusion: We obtained supporting information of the associations between cinacalcet response and CASR polymorphisms. CASR single nucleotide polymorphisms (SNPs rs1802757, rs1042636, and haplotypes of rs1042636, rs10190, and rs1802757 were significantly associated with cinacalcet response variance. Keywords: CASR, calcium sensing receptor, SHPT, genetic polymorphisms, haplotype, single nucleotide polymorphisms

  2. A patient-specific model of the biomechanics of hip reduction for neonatal Developmental Dysplasia of the Hip: Investigation of strategies for low to severe grades of Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Huayamave, Victor; Rose, Christopher; Serra, Sheila; Jones, Brendan; Divo, Eduardo; Moslehy, Faissal; Kassab, Alain J; Price, Charles T

    2015-07-16

    A physics-based computational model of neonatal Developmental Dysplasia of the Hip (DDH) following treatment with the Pavlik Harness (PV) was developed to obtain muscle force contribution in order to elucidate biomechanical factors influencing the reduction of dislocated hips. Clinical observation suggests that reduction occurs in deep sleep involving passive muscle action. Consequently, a set of five (5) adductor muscles were identified as mediators of reduction using the PV. A Fung/Hill-type model was used to characterize muscle response. Four grades (1-4) of dislocation were considered, with one (1) being a low subluxation and four (4) a severe dislocation. A three-dimensional model of the pelvis-femur lower limb of a representative 10 week-old female was generated based on CT-scans with the aid of anthropomorphic scaling of anatomical landmarks. The model was calibrated to achieve equilibrium at 90° flexion and 80° abduction. The hip was computationally dislocated according to the grade under investigation, the femur was restrained to move in an envelope consistent with PV restraints, and the dynamic response under passive muscle action and the effect of gravity was resolved. Model results with an anteversion angle of 50° show successful reduction Grades 1-3, while Grade 4 failed to reduce with the PV. These results are consistent with a previous study based on a simplified anatomically-consistent synthetic model and clinical reports of very low success of the PV for Grade 4. However our model indicated that it is possible to achieve reduction of Grade 4 dislocation by hyperflexion and the resultant external rotation. Copyright © 2015 Elsevier Ltd. All rights reserved.

  3. Effect of surgery on cardiovascular risk factors in mild primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Bollerslev, Jens; Rosen, Thord; Mollerup, Charlotte

    2009-01-01

    CONTEXT: Mild primary hyperparathyroidism (pHPT) seems to have a good prognosis, and indications for active treatment (surgery) are widely discussed. The extraskeletal effects of PTH, such as insulin resistance, arterial hypertension, and cardiovascular (CV) risk, may however be reversible...

  4. Effects of parathyroidectomy versus observation on the development of vertebral fractures in mild primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Lundstam, Karolina; Heck, Ansgar; Mollerup, Charlotte

    2015-01-01

    CONTEXT: Mild primary hyperparathyroidism (PHPT) is a common disease especially in middle-aged and elderly women. The diagnosis is frequently made incidentally and treatment strategies are widely discussed. OBJECTIVE: To study the effect of parathyroidectomy (PTX) compared with observation (OBS...

  5. Skeletal effects of vitamin D deficiency among patients with primary hyperparathyroidism.

    Science.gov (United States)

    Lee, J H; Kim, J H; Hong, A R; Kim, S W; Shin, C S

    2017-02-07

    Little is known about the association between vitamin D deficiency and the skeletal phenotypes in primary hyperparathyroidism (PHPT) patients. A low 25-hydroxyvitamin D level was associated with a low bone mineral density and deteriorated hip geometry in women with PHPT in an Asian population where vitamin D deficiency is prevalent.

  6. Smoking and hyperparathyroidism in patients with end-stage renal disease (ESRD)

    NARCIS (Netherlands)

    G.L. Tripepi (Giovanni); F.U.S. Mattace Raso (Francesco); P. Pizzini (Patrizia); S. Cutrupi (Sebastiano); J.C.M. Witteman (Jacqueline); C. Zoccali (Carmine); F. Mallamaci (Francesca)

    2012-01-01

    textabstractBackground and methods: Smoking is associated with hyperparathyroidism in the elderly general population and nicotine, the main component of tobacco smoke, stimulates PTH release in experimental models. Although smoking is a persisting problem in patients with endstage renal disease (ESR

  7. Rational use of localization studies in the management of primary hyperparathyroidism

    Institute of Scientific and Technical Information of China (English)

    美国,康涅狄格州,纽黑文市,耶鲁大学医学院外科

    2016-01-01

    [Summary] Parathyroidectomy is the first choice for primary hyperparathyroidism(1 HPTH). With the help of pre-operation imaging modalities, focused parathyroidectomy is now widely used. However, the imaging tests play no role in the diagnosis of 1 HPTH, and negative imaging results should not preclude the pursuit of a surgical cure.

  8. Secondary hyperparathyroidism and mortality in hip fracture patients compared to a control group from general practice

    DEFF Research Database (Denmark)

    Madsen, Christian Medom; Jørgensen, Henrik Løvendahl; Lind, Bent

    2012-01-01

    INTRODUCTION: Previously, little attention has been paid as to how disturbances in the parathyroid hormone (PTH)-calcium-vitamin D-axis, such as secondary hyperparathyroidism (SHPT), relate to mortality amongst hip fracture patients. This study aimed to (1) determine if SHPT is associated...

  9. Involvement of the MEN1 gene locus in familial isolated hyperparathyroidism

    DEFF Research Database (Denmark)

    Villablanca, Andrea; Wassif, Wassif S; Birket-Smith, Niels Thomas

    2002-01-01

    Familial isolated hyperparathyroidism (FIHP) is a hereditary disorder characterised by uni- or multiglandular parathyroid disease. A subset of families are likely to be genetic variants of other familial tumour syndromes in which PHPT is the main feature, for example multiple endocrine neoplasia...

  10. Plasmacytoma Mimicking Mediastinal Parathyroid Tumour in a Patient with Primary Hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Jubbin Jagan Jacob

    2007-04-01

    Full Text Available The association of monoclonal gammopathies with primary hyperparathyroidism is well documented. Many case reports have documented the coexistence of primary hyperparathyroidism and multiple myeloma. The cause of this relationship is not known. We report the case of a 49-year-old gentleman who was treated for primary hyperparathyroidism. His initial preoperative nuclear scan had shown persistent activity and retention of tracer in the retrosternal region in addition to the discrete hot spot in the region of the lower pole of the left lobe of the thyroid. During surgery, the enlarged left inferior parathyroid gland was removed. In addition, the retrosternal area was also explored and found to be normal. Ten months later, he developed a mass in the region of the manubrium sternii which was proven to be a plasmacytoma. We review the literature for similar cases and suggest hypotheses for a possible association. In conclusion, coexisting plasma cell dyscrasias including plasmacytoma should be considered in patients with primary hyperparathyroidism.

  11. Cinacalcet versus Parathyroidectomy in the Treatment of Secondary Hyperparathyroidism Post Renal Transplantation

    Directory of Open Access Journals (Sweden)

    Soliman Amin R.

    2016-09-01

    Full Text Available Background. Persistent hyperparathyroidism (HPT with hypercalcemia is prevalent after transplant and is considered a risk factor for progressive bone loss and fractures and vascular calcification, as well as the development of tubulointerstitial calcifications of renal allografts and graft dysfunction. The subtotal parathyroidectomy is the standard treatment, although currently it has been replaced by the calcimimetic cinacalcet.

  12. Nosocomial Infections in Neonatal Intensive Care Units

    OpenAIRE

    2013-01-01

    Neonates, especially prematures, requiring care in Intensive Care Unit are a highly vulnerable population group at increased risk for nosocomial infections. In recent decades become one of the leading causes of morbidity and mortality in the Neonatal Intensive Care Unit. Aim: Highlighting the severity of nosocomial infections for hospitalized infants and the imprinting of risk factors that affects their development. Material-Methods: Searched for studies published in international scientific ...

  13. Neonatal Seizures. Advances in Mechanisms and Management.

    OpenAIRE

    2014-01-01

    Seizures occur in approximately 1–5 per 1,000 live births, and are among the most common neurologic conditions managed by a neonatal neurocritical care service. There are several, age-specific factors that are particular to the developing brain, which influence excitability and seizure generation, response to medications, and impact of seizures on brain structure and function. Neonatal seizures are often associated with serious underlying brain injury such as hypoxia-ischemia, stroke or hemor...

  14. Supra-Acetabular Brown Tumor due to Primary Hyperparathyroidism Associated with Chronic Renal Failure

    Directory of Open Access Journals (Sweden)

    Rosaria M. Ruggeri

    2010-01-01

    Full Text Available A 63-year-old woman presented to the Orthopedic Unit of our hospital complaining of right hip pain of 6 months'duration associated with a worsening limp. Her past medical history included chronic renal insufficiency. Physical examination revealed deep pain in the iliac region and severe restriction of the right hip's articular function in the maximum degrees of range of motion. X-rays and CT scan detected an osteolytic and expansive lesion of the right supra-acetabular region with structural reabsorption of the right iliac wing. 99mTc-MDP whole-body bone scan showed an abnormal uptake in the right iliac region. Bone biopsy revealed an osteolytic lesion with multinucleated giant cells, indicating a brown tumor. Serum intact PTH was elevated (1020 pg/ml; normal values, 12 62 pg/ml, but her serum calcium was normal (total = 9.4 mg/dl, nv 8.5–10.5; ionized = 5.0 mg/dl, nv 4.2–5.4 due to the coexistence of chronic renal failure. 99mTc-MIBI scintigraphy revealed a single focus of sestamibi accumulation in the left retrosternal location, which turned out to be an intrathoracic parathyroid adenoma at surgical exploration. After surgical removal of the parathyroid adenoma, PTH levels decreased to 212 pg/ml. Three months after parathyroidectomy, the imaging studies showed complete recovery of the osteolytic lesion, thus avoiding any orthopedic surgery. This case is noteworthy because (1 primary hyperparathyroidism was not suspected due to the normocalcemia, likely attributable to the coexistence of chronic renal failure; and (2 it was associated with a brown tumor of unusual location (right supra-acetabular region.

  15. Evaluation of clinical manifestations and laboratory findings of primary hyperparathyroidism in Imam Hospital (1988-1998

    Directory of Open Access Journals (Sweden)

    Alam Salimi M

    2002-11-01

    Full Text Available Introduction: Clinical manifestations of primary hyper-para-thyroidism (pHPT had been dramatically changed during last 25 years. Evaluation of changes in clinical findings was the aim of this study. Materials and methods: In a retrospective, descriptive case series, patients' records of all 47 pHPT (44 females, 3 males from 1988 till 1998 were studied. Patients’ clinical presentations, signs and symptoms, laboratory and radiologic findings were reviewed and the results were compared with 34 patients' studied during 1978-1987. Serum Ca>10.5 mg/dl with increased or high normal PTH were the diagnostic criteria of pHPT. Results: Patients’ age range was 11-70 and mean ±SD was 38±16 years, with a female to male ratio of 14:1. 57 percent of the patients had bone pain and muscle weakness, 12 percent were asymptomatic, 10 percent had pathologic fractures, 8 percent had renal stones, 8 percent had symptoms of hypercalcemia, and 2 percent had giant cell lesion. The mean±SD of serum calcium was 11.48±1.16 mg/dl, phosphorus was 2.4±0.6 mg/dl and 24-h urinary Ca was 294±197 mg. Serum PTH was increased from 1.5 to 500 folds. The frequency of single adenoma in right inferior, left inferior, and left superior gland were 43 percent, 30 percent, and 13 percent respectively. Conclusion: In the study 12 percent of patients were asymptomatic whereas there was no asymptomatic case in the previous study. Prevalence of severe bone disease and the interval between onset of symptoms and diagnosis was also reduced. According to this study detection of pHPT in asymptomatic phase remarkably increased.

  16. The OPTIMA Study: Assessing a New Cinacalcet (Sensipar/Mimpara) Treatment Algorithm for Secondary Hyperparathyroidism

    Science.gov (United States)

    Messa, Piergiorgio; Macário, Fernando; Yaqoob, Magdi; Bouman, Koen; Braun, Johann; von Albertini, Beat; Brink, Hans; Maduell, Francisco; Graf, Helmut; Frazão, João M.; Bos, Willem Jan; Torregrosa, Vicente; Saha, Heikki; Reichel, Helmut; Wilkie, Martin; Zani, Valter J.; Molemans, Bart; Carter, Dave; Locatelli, Francesco

    2008-01-01

    Background and objectives: Cinacalcet, a novel calcimimetic, targets the calcium-sensing receptor to lower parathyroid hormone (PTH), calcium, and phosphorus levels in dialysis patients with secondary hyperparathyroidism (SHPT). This study compared the efficacy of a cinacalcet-based regimen with unrestricted conventional care (vitamin D and phosphate binders) for achieving the stringent National Kidney Foundation Kidney Disease Outcomes Quality Initiative (KDOQI) targets for dialysis patients. Study design: In this multicenter, open-label study, hemodialysis patients with poorly controlled SHPT were randomized to receive conventional care (n = 184) or a cinacalcet-based regimen (n = 368). Doses of cinacalcet, vitamin D sterols, and phosphate binders were adjusted during a 16-wk dose-optimization phase with the use of algorithms that allowed cinacalcet to be used with adjusted doses of vitamin D. The primary end point was the proportion of patients with mean intact PTH ≤300 pg/ml during a 7-wk efficacy assessment phase. Results: A higher proportion of patients receiving the cinacalcet-based regimen versus conventional care achieved the targets for PTH (71% versus 22%, respectively; P < 0.001), Ca × P (77% versus 58%, respectively; P < 0.001), calcium (76% versus 33%, respectively; P < 0.001), phosphorus (63% versus 50%, respectively; P = 0.002), and PTH and Ca × P (59% versus 16%, respectively, P < 0.001), and allowed a 22% reduction in vitamin D dosage in patients receiving vitamin D at baseline. Achievement of targets was greatest in patients with less severe disease (intact PTH range, 300 to 500 pg/ml) and the cinacalcet dose required was lower in these patients (median = 30 mg/d). Conclusions: Compared with conventional therapy, a cinacalcet-based treatment algorithm increased achievement of KDOQI treatment targets in dialysis patients in whom conventional therapy was no longer effective in controlling this disease. PMID:18178780

  17. Parathyroid scintigraphy findings in chronic kidney disease patients with recurrent hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Hindie, Elif [Universite Paris 7, Nuclear Medicine, Hopital Saint Louis, Paris (France); Hopital Saint-Louis, Service de Medecine Nucleaire, Paris (France); Zanotti-Fregonara, Paolo; Keller, Isabelle [Universite Paris VI, Nuclear Medicine, Hopital Saint Antoine, Paris (France); Just, Pierre-Alexandre; Toubert, Marie-Elisabeth; Moretti, Jean-Luc [Universite Paris 7, Nuclear Medicine, Hopital Saint Louis, Paris (France); Sarfati, Emile [Universite Paris 7, Endocrine Surgery, Hopital Saint Louis, Paris (France); Melliere, Didier [Universite Paris XII, Endocrine Surgery, Hopital Henri Mondor, Creteil (France); Jeanguillaume, Christian [Universite d' Angers, Centre Paul Papin, Angers (France); Urena-Torres, Pablo [Clinique du Landy, Service de Nephrologie-Dialyse, Saint Ouen (France)

    2010-03-15

    Parathyroidectomy (PTX), either subtotal or total with forearm autografting, is a well-established treatment for refractory renal hyperparathyroidism (RHPT). However, 20-30% of patients develop persistent or recurrent disease. Obtaining accurate localization before reoperation is difficult. The study group comprised 21 consecutive adult patients (18 undergoing haemodialysis and 3 with a renal graft) imaged using {sup 99m}Tc-sestamibi/{sup 123}I subtraction scintigraphy. Of the 21 patients, 12 had undergone one previous PTX and the other 9 between two and four parathyroid operations. All patients had symptoms and signs of severe RHPT. The mean serum PTH level was 1,142 pg/ml. {sup 99m}Tc-Sestamibi and {sup 123}I images were recorded simultaneously. Imaging views comprised a planar view of the neck and mediastinum, followed by a magnified pinhole view over the thyroid bed area. If parathyroid ectopy was detected, SPECT or SPECT-CT was performed. The forearm was imaged in case of autograft. Parathyroid scintigraphy was negative in one patient and positive in the other 20 (sensitivity 95.2%). One patient had uptake corresponding to two unresected parathyroid glands. Recurrence at the site of the partially resected gland or autograft was seen in 11 patients. However, six of them had a second {sup 99m}Tc-sestamibi focus corresponding to a supernumerary parathyroid gland. Seven other patients had a supernumerary parathyroid gland as the sole cause of relapse. Three of the supernumerary glands showed major ectopy (intrathyroidal, low mediastinal, undescended within the vagus nerve). One patient had parathyromatosis with multiple parathyroid nodules scattered over the left side of the neck. Reoperation was possible in 13 patients, with no false-positive findings. Many patients referred with the hypothesis of hyperplasia of a subtotally resected parathyroid gland or autograft were found to harbour a supernumerary parathyroid gland missed at the initial surgery. (orig.)

  18. Clinical and Pathological Studies on Severe Parathyroid Hyperplasia in Uremic Patients with Secondary Hyperparathyroidism

    Institute of Scientific and Technical Information of China (English)

    SunB; WangXY

    2002-01-01

    Objective To evaluate clinical significance of parathyroid proliferation in secondary hyperpar athyroidism(SHPT).Methods The specimens of parathyroid were taken from 7 patients with SHPT and resistance to medical therapy.The histological sections were routinely stained with hematoxylin-eoxin(HE) stain,and according to macroscopical and microscopical examinations,divided into two types:diffuse type (D-type) with a normal lobular constitution and nodular type(N-type).The sum of parathyroid cells under HE stain were calculated by computer image analysis system.Results There was 16.1 times increase in the weight of parathyroid of CRF patients with SHPT and 1.86 times increase in sum of parathyroid cells.The proliferation of N-type parathyroid was especially noticeable.Conclusion We suggest that D-type hyperplasia parathryoid should be selected in autotransplantation,expecially quite small piece,in order to prevent symtom recurrence of SHPT.

  19. {sup 131}I treatment for thyroid cancer and risk of developing primary hyperparathyroidism: a cohort study

    Energy Technology Data Exchange (ETDEWEB)

    Lin, Chien-Mu [Taipei Medical University - Shuang Ho Hospital, Department of Nuclear Medicine, Taipei (China); Taipei Medical University, Department of Radiology, College of Medicine, Taipei (China); Doyle, Pat [London School of Hygiene and Tropical Medicine, Faculty of Epidemiology and Population Health, London (United Kingdom); Tsan, Yu-Tse [National Taiwan University College of Public Health, Institute of Occupational Medicine and Industrial Hygiene, Taipei (China); Taichung Veterans General Hospital, Department of Emergency Medicine, Taichung (China); Chung Shan Medical University, School of Medicine, Taichung (China); Lee, Chang-Hsing [Ton Yen General Hospital, Department of Occupational Medicine, Hsinchu County (China); Wang, Jung-Der [National Taiwan University College of Public Health, Institute of Occupational Medicine and Industrial Hygiene, Taipei (China); National Cheng Kung University College of Medicine, Department of Public Health, Tainan (China); Chen, Pau-Chung [National Taiwan University College of Public Health, Institute of Occupational Medicine and Industrial Hygiene, Taipei (China); National Taiwan University College of Public Health, Department of Public Health, Taipei (China); National Taiwan University College of Medicine and Hospital, Department of Environmental and Occupational Medicine, Taipei (China); Collaboration: Health Data Analysis in Taiwan (hDATa) Research Group

    2014-02-15

    To evaluate the association between {sup 131}I therapy for thyroid cancer and risk of developing primary hyperparathyroidism. This was a nationwide population-based cohort study of patients with thyroid cancer diagnosed during the period 1997-2008. The data were obtained from the Taiwan National Health Insurance Research dataset. The cumulative {sup 131}I dose in each patient was calculated. Hazard ratios (HRs) were calculated using a proportional hazards model to estimate the effect of {sup 131}I therapy on the risk of developing primary hyperparathyroidism in the cohort. A total of 8,946 patients with thyroid cancer were eligible for the final analysis. Among these patients, 8 developed primary hyperparathyroidism during the follow-up period that represented 38,248 person-years giving an incidence rate of 20.9 per 10{sup 5} person-years. {sup 131}I was used in the treatment of 6,153 patients (68.8 %) with a median cumulative dose of 3.7 GBq. The adjusted HRs were 0.21 (95% CI 0.02-1.86) and 0.46 (95% CI 0.10-2.10) for those receiving a cumulative {sup 131}I dose of 0.1-3.6 GBq and ≥3.7 GBq, respectively, compared to no therapy. The risk of developing primary hyperparathyroidism did not increase with increasing {sup 131}I dose (test for trend p = 0.51). No interaction was found between {sup 131}I dose and age (p = 0.94) or {sup 131}I dose and sex (p = 0.99). {sup 131}I treatment for thyroid cancer did not increase risk of primary hyperparathyroidism during a 10-year follow-up in this study population. Further research with a longer follow-up period is needed to assess late adverse effects beyond 10 years. (orig.)

  20. Prevalence of primary hyperparathyroidism in a referred sample of fibromyalgia patients.

    Science.gov (United States)

    Ferrari, Robert; Russell, Anthony Science

    2015-07-01

    The objective of this study is to determine the prevalence of primary hyperparathyroidism in a referred sample of fibromyalgia patients. Consecutively, referred patients with confirmed fibromyalgia (FM group) had measurements of serum levels of vitamin D, alkaline phosphatase, total calcium, magnesium, phosphate, creatinine, total protein, albumin, and parathyroid hormone. The same measurements were also conducted in a group of patients with widespread pain (WP group) who did not meet the 2010 Modified ACR criteria for fibromyalgia and a group with localized musculoskeletal pain (MSK group). A case of primary hyperparathyroidism was defined as a subject whose results showed any of the following: (1) parathyroid hormone levels above 6.8 pmol/L; (2) an ionized calcium above 1.25 mmol/L; or (3) both elevated, in the presence of normal range creatinine, alkaline phosphatase, vitamin D, phosphate, and magnesium. The mean age and the proportion of subjects who met the case definition of primary hyperparathyroidism were calculated for all groups. There were 125 subjects in the FM group, 127 in the WP group, and 138 in the MSK group. The prevalence rates of primary hyperparathyroidism were 6.4, 5.5, and 6.1%, respectively, for these groups. Comparison of these prevalence rates to published figures for general clinical and non-clinical populations reveals no differences. The prevalence of primary hyperparathyroidism in fibromyalgia patients is not different than that in other patients with WP or those with localized pain, nor is it likely different than that seen in the general population.

  1. Cinacalcet HCl, an oral calcimimetic agent for the treatment of secondary hyperparathyroidism in hemodialysis and peritoneal dialysis: a randomized, double-blind, multicenter study.

    Science.gov (United States)

    Lindberg, Jill S; Culleton, Bruce; Wong, Gordon; Borah, Michael F; Clark, Roderick V; Shapiro, Warren B; Roger, Simon D; Husserl, Fred E; Klassen, Preston S; Guo, Matthew D; Albizem, Moetaz B; Coburn, Jack W

    2005-03-01

    Management of secondary hyperparathyroidism is challenging with traditional therapy. The calcimimetic cinacalcet HCl acts on the calcium-sensing receptor to increase its sensitivity to calcium, thereby reducing parathyroid hormone (PTH) secretion. This phase 3, multicenter, randomized, placebo-controlled, double-blind study evaluated the efficacy and safety of cinacalcet in hemodialysis (HD) and peritoneal dialysis (PD) patients with PTH > or =300 pg/ml despite traditional therapy. A total of 395 patients received once-daily oral cinacalcet (260 HD, 34 PD) or placebo (89 HD, 12 PD) titrated from 30 to 180 mg to achieve a target intact PTH (iPTH) level of or =30% reduction in iPTH from baseline (65 versus 13%), and proportion of patients with > or =20, > or =40, or > or =50% reduction from baseline. Cinacalcet had comparable efficacy in HD and PD patients; 50% of PD patients achieved a mean iPTH < or =300 pg/ml. Cinacalcet also significantly reduced serum calcium, phosphorus, and Ca x P levels compared with control treatment. The most common side effects, nausea and vomiting, were usually mild to moderate in severity and transient. Once-daily oral cinacalcet was effective in rapidly and safely reducing PTH, Ca x P, calcium, and phosphorus levels in patients who received HD or PD. Cinacalcet offers a new therapeutic option for controlling secondary hyperparathyroidism in patients with chronic kidney disease on dialysis.

  2. Mechanical ventilation of the premature neonate.

    Science.gov (United States)

    Brown, Melissa K; DiBlasi, Robert M

    2011-09-01

    Although the trend in the neonatal intensive care unit is to use noninvasive ventilation whenever possible, invasive ventilation is still often necessary for supporting pre-term neonates with lung disease. Many different ventilation modes and ventilation strategies are available to assist with the optimization of mechanical ventilation and prevention of ventilator-induced lung injury. Patient-triggered ventilation is favored over machine-triggered forms of invasive ventilation for improving gas exchange and patient-ventilator interaction. However, no studies have shown that patient-triggered ventilation improves mortality or morbidity in premature neonates. A promising new form of patient-triggered ventilation, neurally adjusted ventilatory assist (NAVA), was recently FDA approved for invasive and noninvasive ventilation. Clinical trials are underway to evaluate outcomes in neonates who receive NAVA. New evidence suggests that volume-targeted ventilation modes (ie, volume control or pressure control with adaptive targeting) may provide better lung protection than traditional pressure control modes. Several volume-targeted modes that provide accurate tidal volume delivery in the face of a large endotracheal tube leak were recently introduced to the clinical setting. There is ongoing debate about whether neonates should be managed invasively with high-frequency ventilation or conventional ventilation at birth. The majority of clinical trials performed to date have compared high-frequency ventilation to pressure control modes. Future trials with premature neonates should compare high-frequency ventilation to conventional ventilation with volume-targeted modes. Over the last decade many new promising approaches to lung-protective ventilation have evolved. The key to protecting the neonatal lung during mechanical ventilation is optimizing lung volume and limiting excessive lung expansion, by applying appropriate PEEP and using shorter inspiratory time, smaller tidal

  3. Effectiveness of cinacalcet in patients with recurrent/persistent secondary hyperparathyroidism following parathyroidectomy: results of the ECHO study

    DEFF Research Database (Denmark)

    Zitt, Emanuel; Rix, Marianne; Ureña Torres, Pablo

    2010-01-01

    Background. Progressive secondary hyperparathyroidism (sHPT) is characterized by parathyroid gland hyperplasia which may ultimately require parathyroidectomy (PTX). Although PTX is generally a successful treatment for those patients subjected to surgery, a significant proportion develops recurrent...

  4. Effectiveness of cinacalcet in patients with recurrent/persistent secondary hyperparathyroidism following parathyroidectomy: results of the ECHO study

    DEFF Research Database (Denmark)

    Zitt, Emanuel; Rix, Marianne; Ureña Torres, Pablo

    2011-01-01

    Progressive secondary hyperparathyroidism (sHPT) is characterized by parathyroid gland hyperplasia which may ultimately require parathyroidectomy (PTX). Although PTX is generally a successful treatment for those patients subjected to surgery, a significant proportion develops recurrent s...

  5. Serum phosphorus reduction in dialysis patients treated with cinacalcet for secondary hyperparathyroidism results mainly from parathyroid hormone reduction

    DEFF Research Database (Denmark)

    Zitt, Emanuel; Fouque, Denis; Jacobson, Stefan H

    2013-01-01

    The calcimimetic cinacalcet lowers parathyroid hormone (PTH), calcium (Ca) and phosphorus (P) in dialysis patients with secondary hyperparathyroidism (SHPT). We explored serum P changes in dialysis patients treated with cinacalcet, while controlling for vitamin D sterol and phosphate binder (PB...

  6. No beneficial effects of vitamin D supplementation on muscle function or quality of life in primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Rolighed, Lars; Rejnmark, Lars; Sikjaer, Tanja

    2014-01-01

    Context: Impairments of muscle function and strength in patients with primary hyperparathyroidism (PHPT) are rarely addressed although decreased muscle function may contribute to increased fracture risk. Objective: We aimed to assess changes in muscle strength, muscle function, postural stability...

  7. Outcome of total parathyroidectomy and autotransplantation as treatment of secondary and tertiary hyperparathyroidism in children and adults.

    NARCIS (Netherlands)

    Kievit, A.J.; Tinnemans, J.G.; Idu, M.M.; Groothoff, J.W.; Surachno, S.; Aronson, D.C.

    2010-01-01

    BACKGROUND: Treatment safety and effectiveness of total parathyroidectomy and autotransplantation for secondary and tertiary hyperparathyroidism have been extensively proven in adults; the evidence for children, however, is scarce. Children and adolescents cannot simply be seen as young adults in th

  8. Myocardial perfusion defects and the left ventricular ejection fraction disclosed by scintigraphy in patients with primary hyperparathyroidism

    DEFF Research Database (Denmark)

    Ogard, Christina Gerlach; Søndergaard, Susanne Bonnichsen; Jakobsen, Henrik

    2005-01-01

    Patients with primary hyperparathyroidism (PHPT) have increased risk of cardiovascular disease. For patients undergoing preoperative parathyroid imaging with 99mTc-sestamibi single photon emission computed tomography (SPECT), we combined cervical SPECT and gated cardiac SPECT to achieve informati...

  9. The cost-effectiveness of sestamibi scanning compared to bilateral neck exploration for the treatment of primary hyperparathyroidism.

    Science.gov (United States)

    Ruda, James; Stack, Brendan C; Hollenbeak, Christopher S

    2004-08-01

    This article presents a cost-effectiveness analysis to determine whether preoperative imaging with Tc99m-sestamibi for detection and treatment of solitary adenomas associated with primary hyperparathyroidism is cost-effective compared with routine bilateral neck exploration.

  10. Neonatal muscular manifestations in mitochondrial disorders.

    Science.gov (United States)

    Tulinius, Már; Oldfors, Anders

    2011-08-01

    During the last decade rapid development has occurred in defining nuclear gene mutations causing mitochondrial disease. Some of these newly defined gene mutations cause neonatal or early infantile onset of disease, often associated with severe progressive encephalomyopathy combined with other multi-organ involvement such as cardiomyopathy or hepatopathy and with early death. Findings suggesting myopathy in neonates are hypotonia, muscle weakness and wasting, and arthrogryposis. We aim to describe the clinical findings of patients with mitochondrial disease presenting with muscular manifestations in the neonatal period or in early infancy and in whom the genetic defect has been characterized. The majority of patients with neonatal onset of mitochondrial disease have mutations in nuclear genes causing dysfunction of the mitochondrial respiratory chain, leading to defective oxidative phosphorylation.

  11. Craniofacial brown tumor as a result of secondary hyperparathyroidism in chronic renal disease patient: A rare entity

    Science.gov (United States)

    Verma, Pradhuman; Verma, Kanika Gupta; Verma, Dinesh; Patwardhan, Nitin

    2014-01-01

    Brown tumors are erosive bony lesions caused by rapid osteoclastic activity and peritrabecular fibrosis due to primary or secondary hyperparathyroidism resulting in a local destructive phenomenon. The differential diagnosis based on histological examination is only presumptive. Clinical, radiological and laboratory data are necessary for definitive diagnosis. Here, we report a very rare case of brown tumor involving maxilla and mandible, which is the result of secondary hyperparathyroidism in 30-year-old female patient with chronic renal disease. PMID:25328310

  12. Neonatal septic arthritis.

    Science.gov (United States)

    Halder, D; Seng, Q B; Malik, A S; Choo, K E

    1996-09-01

    Neonatal septic arthritis has always been considered as separate from its counterpart in older children. The condition is uncommon but serious. Affected neonates usually survive, but with permanent skeletal deformities. Ten cases of neonatal septic arthritis were diagnosed between January 1989 and December 1993 in the neonatal intensive care units of two referral hospitals in the state of Kelantan, Malaysia. All except one neonate was born prematurely. The mean age of presentation was 15.6 days. Joint swelling (10/10), increased warmth (7/10) and erythema of the overlying skin (7/10) were the common presenting signs. Vague constitutional symptoms preceded the definitive signs of septic arthritis in all cases. The total white cell counts were raised with shift to the left. The knee (60%) was not commonly affected, followed by the hip (13%) and ankle (13%). Three neonates had multiple joint involvement. Coexistence of arthritis with osteomyelitis was observed in seven neonates. The commonest organism isolated was methicillin resistant Staphylococcus aureus (9/10). Needle aspiration was performed in nine neonates and one had incision with drainage. Follow up data was available for five neonates and two of these had skeletal morbidity. Early diagnosis by frequent examination of the joints, prompt treatment and control of nosocomial infection are important for management.

  13. Enterotoxemia in neonatal calves.

    Science.gov (United States)

    Fleming, S

    1985-11-01

    The incidence, bacterial characteristics, disease syndromes, diagnosis, treatment, and prevention of enterotoxemia of neonatal calves caused by Clostridium perfringens (Types A, B, C, D, and E) are reviewed.

  14. 探讨机械通气下应用肌松剂治疗重型新生儿破伤风的疗效%Curative Effect Under Mechanical Ventilation Applied Muscle Relaxants for the Treatment of Severe Neonatal Tetanus

    Institute of Scientific and Technical Information of China (English)

    许英

    2015-01-01

    目的:探讨机械通气下应用肌松剂治疗对重型新生儿破伤风的疗效。方法选择30例重型破伤风新生儿,随机分为观察组和对照组各15例。对照组进行综合治疗,观察组于机械通气下应用肌松剂治疗。结果观察组无患者死亡,死亡率低于对照组53.3%(P<0.05);观察组并发症发生情况优于对照组(P<0.05)。结论对重型破伤风新生儿于机械通气下应用肌松剂治疗,有助于改善患儿并发症的产生,降低重型新生儿破伤风的死亡率。%Objective To study the application of nondepolarizing agent under mechanical ventilation in treatment of severe neonatal tetanus.Methods 30 cases of heavy neonatal tetanus,were randomly divided into observation group and control group(n=15). Comprehensive treatment for control group and observation group used muscle relaxants under mechanical ventilation treatment.Results Observation group had no patients died,was lower than the control group 53.3%(P< 0.05). Observation group complications situation is better than that of control group(P< 0.05).Conclusion The heavy neonatal tetanus under mechanical ventilation and muscle relaxants treatment help children improve complications,reduce the mortality of severe neonatal tetanus.

  15. 重症胎粪吸入综合征新生儿危险因素及并发症%Risk factors and complications of severe meconium aspiration syndrome in neonates

    Institute of Scientific and Technical Information of China (English)

    冯艺; 熊涛; 屈艺; 伍金林; 母得志

    2013-01-01

    目的 分析胎粪吸入综合征(MAS)新生儿的临床资料,为早期发现重症MAS及预防并发症提供依据.方法 对四川大学华西第二医院新生儿科2006年12月至2011年12月收治的107例确诊为MAS新生儿的临床资料进行回顾性分析.根据是否需要呼吸机辅助通气(包括采用持续呼吸道正压通气及有创通气)分为重症MAS组(52例)、非重症MAS组(55例),采用SPSS 15.0软件进行统计分析,比较2组患儿一般情况、母儿孕期健康状况及并发症.结果 2组患儿在出生1 min、5 min、10 min Apgar评分,胎儿宫内窘迫,出生时气管插管吸取胎粪之间的差异均有统计学意义.并发症中以缺氧缺血性脑病(HIE)(61.7%)、心肌损伤(43.0%)、代谢紊乱(41.1%)和颅内出血(20.6%)最常见.重症MAS组患儿中,HIE、代谢紊乱、DIC、颅内出血、肺出血、心肌损伤、肾功能损伤等并发症发生率均高于非重症MAS组,尚未发现重症MAS与母亲孕期并发症、生产方式如剖宫产等相关.结论 重症MAS发生与胎儿宫内窘迫、出生时窒息相关.MAS尤其是重症MAS易合并多器官系统损伤,应加强监测.%Objective To provide the clinical evidence for early diagnosis of meconium aspiration syndrome (MAS) and to prevent its complications.Methods One hundred and seven cases of neonates diagnosed with MAS treated in Department of Neonatology,West China Second Hospital,Sichuan University,from Dec.2006 to Dec.2011were analyzed retrospectively.They were assigned into severe MAS group (52 cases) and non-severe MAS group (55 cases) according to whether need mechanical ventilation(including positive end-expiratory pressure and invasive ventilation).Comparisons were carried out with SPSS 15.0,including the general situation of the newborns,obstetric characteristics and complications of both groups.Results There were significant differences in 1 min,5 min and 10 min Apgar score,intrauterine distress

  16. Fetal/neonatal alloimmune thrombocytopenia.

    Science.gov (United States)

    Sachs, Ulrich J

    2013-01-01

    In fetal/neonatal alloimmune thrombocytopenia (FNAIT), antibodies against paternal antigens present on fetal platelets are produced by the mother. These antibodies will cross the placenta and can cause thrombocytopenia of the unborn. One severe consequence of thrombocytopenia is intracranial bleeding which may lead to death or severe sequelae. FNAIT index cases in one family are usually detected at birth only since antenatal screening programmes have not been installed so far. Subsequent pregnancies of immunized mothers may require special diagnostic and prophylactic interventions, and interdisciplinary counselling and treatment involving obstetricians, pediatricians and immunohematologists may prove useful for optimized care. This short review covers pathogenesis, clinical presentation, diagnostic, and therapeutic options in FNAIT.

  17. Recurrence of Hyperparathyroid Hypercalcemia in a Patient With the HRPT-2 Mutation and a Previous Parathyroid Carcinoma in Hyperparathyroidism-Jaw Tumor Syndrome

    Directory of Open Access Journals (Sweden)

    Mele

    2016-04-01

    Full Text Available Introduction Cancer in the parathyroid gland is rare, but parathyroid cancer is occasionally seen in relation to genetic abnormalities. Due to a limited amount of evidence, the optimal handling of these cases is not clear. Furthermore, the presence of a malignant parathyroid tumor is rarely known at the time of the initial operation; therefore, re-operations are often necessary. The aim of this study was to present the case of a patient with a previously diagnosed jaw tumor and parathyroid carcinoma that presents as a recurrence of hyperparathyroid hypercalcemia. Case Presentation A 41-year-old patient who was already diagnosed with a parathyroid carcinoma and a jaw tumor caused by a CDC73 mutation, presented with biochemical evidence of increasing parathyroid hormone (PTH and calcium levels after a previous total parathyroidectomy. The patient’s ionized calcium increased to 1.55 mmol/L and PTH increased to 16.0 pmol/L. A previous genetic analysis revealed a mutation in the CDC73 gene. There was no family history of hyperparathyroidism. We performed a sestamibi scintigraphy and an 11-C methionine (MET positron emission tomography (PET scan that showed a recurrence on the left side of the trachea. The patient underwent a third neck operation for the removal of a tumor on the left side of the trachea. The pathology report revealed that the tumor was a lymph node metastasis from the previous parathyroid carcinoma. The patient is currently enrolled in our follow-up regime. Hyperparathyroidism-jaw tumor (HPT-JT syndrome is a rare autosomal dominant disorder characterized by a parathyroid adenoma or carcinoma, fibro-osseous lesions (ossifying fibroma of the mandible and maxilla, and renal cysts and tumors. This autosomal dominant familial cancer syndrome has been reported with a variable and incomplete penetrance, and up to 10% of gene carriers do not show any clinical manifestations. Here we present a patient’s case and discuss the literature

  18. Complications in neonatal surgery.

    Science.gov (United States)

    Escobar, Mauricio A; Caty, Michael G

    2016-12-01

    Neonatal surgery is recognized as an independent discipline in general surgery, requiring the expertise of pediatric surgeons to optimize outcomes in infants with surgical conditions. Survival following neonatal surgery has improved dramatically in the past 60 years. Improvements in pediatric surgical outcomes are in part attributable to improved understanding of neonatal physiology, specialized pediatric anesthesia, neonatal critical care including sophisticated cardiopulmonary support, utilization of parenteral nutrition and adjustments in fluid management, refinement of surgical technique, and advances in surgical technology including minimally invasive options. Nevertheless, short and long-term complications following neonatal surgery continue to have profound and sometimes lasting effects on individual patients, families, and society. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. Observation on the curative effect of monosialotetrahexosylganglioside sodium salt injection for treatment of neonates with moderate and severe hypoxic-ischemic encephalopathy%单唾液酸四己糖神经节苷脂钠注射液治疗中、重度新生儿缺氧缺血性脑病疗效观察

    Institute of Scientific and Technical Information of China (English)

    赵玉萍; 彭晓康

    2012-01-01

    目的:探讨应用单唾液酸四己糖神经节苷脂钠治疗中、重度新生儿缺氧缺血性脑病(HIE)的疗效及对预后的影响,为临床治疗提供参考依据.方法:将92例中、重度HIE患儿随机分为对照组和治疗组,两组均予支持疗法和对症处理,对照组应用胞二磷胆碱,而治疗组应用单唾液酸四己糖神经节苷脂钠,两组方法及疗程相同,最后比较两组NBNA评分.所有病例均于出生后3、6、9、12个月时测定发育商(DQ),对两组临床疗效及预后进行比较.结果:治疗组呼吸改善时间、惊厥消失时间、症状及体征全部消失时间少于对照组;治疗组后遗症发生率低于对照组,治疗组NBNA评分及DQ高于对照组,差异均具有统计学意义(P<0.01).结论:与胞二磷胆碱相比,单唾液酸四己糖神经节苷脂钠治疗中、重度HIE的总有效率有所提高,患儿惊厥易于控制,意识障碍及肌张力等神经症状及体征恢复快,且在改善HIE的预后方面效果显著,可降低后遗症发生率及新生儿死亡率,无任何不良反应,适于临床推广使用.%Objective; To explore the curative effect of monosialotetrahexosylganglioside sodium salt injection for treatment of neonates with moderate and severe hypoxic - ischemic encephalopathy ( HIE) and its impact on prognosis, provide reference for clinical treatment. Methods; A total of 92 neonates with HIE were randomly divided into control group and treatment group, the neonates in the two groups received supporting treatment and symptomatic treatment, then the neonates in control group were treated with cytidine diphosphate choline, the neonates in treatment group were treated with monosialotetrahexosylganglioside sodium salt injection, the methods and courses of treatment in the two groups were the same, NBNA scores in the two groups were compared- Development quotients ( DQs) were measured in all the neonates at 3 , 6, 9, and 12 months after birth; clinical curative

  20. Expression of the calcium receptor CaR in the parathyroid of secondary hyperparathyroidism patients

    Institute of Scientific and Technical Information of China (English)

    王宁宁; 王笑云; 彭韬; 吴宏飞; 胡建明; 赵卫红; 俞香宝

    2004-01-01

    @@ The effects of calcium on parathyroid hormone (PTH) has further discovered in recent years. It has been known that calcium ion concentration in the extracellular fluid is a major determinant of PTH secretion. The relationship between serum intact PTH (iPTH) and calcium ion levels is described by a sigmoidal curve. The calcium concentration that produces half-maximal change in PTH release (the midpoint between maximal and minimal change in PTH release) represents the sensitivity of parathyroid cells to serum calcium. In secondary hyperparathyroidism (SHPT) patients, higher calcium concentrations are needed to suppress PTH secretion, as demonstrated by the PTH-calcium sigmoidal curve. The loss of physiological control over the secretory function and growth of parathyroid tissue in hyperparathyroid disease is still incompletely understood.

  1. Síndrome de hiperparatiroidismo por tumor maxilar Hyperparathyroidism-jaw tumour syndrome

    Directory of Open Access Journals (Sweden)

    L. Barroso

    2008-08-01

    Full Text Available El hiperparatiroidismo tiene con frecuencia manifestaciones óseas, de predomínio facial en algunos pacientes. Los autores describen las manifestaciones en una família de la región central de Portugal, como punto de partida para una revisión de los conocimientos sobre esta entidade clínica todavía poco divulgada y que puede tener como primera manifestación la presentación de tumores faciales.Hyperparathyroidism frequently has bone effects. In one subset of patients, these effects involve mainly facial bones (hyperparathyroidism-jaw tumour syndrome. The authors describe an affected family from central Portugal and discuss the features of this still poorly known disease, which can present initially as a facial tumour.

  2. Localization of primary hyperparathyroidism using {sup 99m}Tc-MIBI scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Yane, Katsunari; Emoto, Mie; Okamoto, Hideyuki; Yuki, Kazue; Hosoi, Hiroshi [Nara Medical Univ., Kashihara (Japan); Miyahara, Hiroshi [Osaka Prefectural Hospital (Japan)

    2003-03-01

    To determine the clinical utility of 99mTc-MIBI scintigraphy (MIBI) in the localization of hyperfunctioning parathyroid lesions, detection of lesions in 14 patients with primary hyperparathyroidism was compared between MIBI, ultrasonography and computed tomography (CT). MIBI (85.7%) and ultrasonography (78.5%) demonstrated higher detection rates than CT (50%). Furthermore, combination of MIBI and ultrasonography (92.8%) increased the detection rate. One case of ectopic parathyroid adenoma was detected only on MIBI. The smallest parathyroid lesion detected using MIBI was a parathyroid adenoma weighing 300 mg. A combination of MIBI and ultrasonography is therefore useful for the localization of primary hyperparathyroidism, and MIBI is particularly useful for detecting ectopic lesions. (author)

  3. Porcine Neonatal Coccidiosis

    Science.gov (United States)

    Sanford, S. E.; Josephson, G. K. A.

    1981-01-01

    Coccidia were identified in intestinal sections from 82 piglets comprising 37 consignments from 34 farms, and represented a yearly increasing incidence in the three years 1978 to 1980. Piglets were primarily from medium to large farms with intensive, continuous-farrowing, confinement-rearing programs. Piglets, usually five days to 15 days old, had yellow, fluid diarrhea, became unthrifty and sometimes died. In six piglets from two farms, a green, adherent, fibrinonecrotic membrane was seen throughout most of the jejunum and ileum. Significant gross lesions were not observed in the other 76 piglets. Moderate to severe villous atrophy of jejunum and ileum was seen histologically. Various asexual and sexual stages of coccidia were seen within parasitophorous vacuoles of villar epithelial cells. Multifocal erosions with necrosis of villar tips and occasionally more diffuse mucosal necrosis with fibrinocellular exudate were seen. Isospora suis oocysts were identified in feces from several weaners from one farm. Amprolium and decoquinate mixed in the sow ration at 1 kg/tonne for three weeks prior to and postfarrowing was moderately successful in stopping outbreaks of neonatal diarrhea associated with coccidiosis. ImagesFigure 1.Figure 2.Figure 3.Figure 4.Figure 5.Figure 6. PMID:7343074

  4. Death in CHARGE syndrome after the neonatal period

    NARCIS (Netherlands)

    Bergman, J. E. H.; Blake, K. D.; Bakker, M. K.; Sarvaas, G. J. du Marchie; Free, R. H.; van Ravenswaaij-Arts, C. M. A.

    2010-01-01

    CHARGE syndrome is a multiple congenital anomaly syndrome that can be life-threatening in the neonatal period. Complex heart defects, bilateral choanal atresia, esophageal atresia, severe T-cell deficiency, and brain anomalies can cause neonatal death. As little is known about the causes of death in

  5. Biochemical profile of erythrocyte membrane of jaundiced neonates.

    Science.gov (United States)

    Mazumder, S; Sarkar, U; Sengupta, D

    2000-01-01

    Studies in newborn humans have demonstrated alteration in the lipid, phospholipid and cholesterol content when compared with age-matched control. Membrane bound (Na+ + K+)ATPase activity is found to be significantly increased in jaundiced neonates. Alteration in membrane permeability characteristics in jaundiced neonates causes severe microenvironmental changes in red blood cell profile.

  6. Does Time of Delivery Influence the Risk of Neonatal Morbidity?

    Science.gov (United States)

    Brookfield, Kathleen F; O'Malley, Katharine; El-Sayed, Yasser Y; Blumenfeld, Yair J; Butwick, Alexander J

    2016-04-01

    To examine whether time of delivery influences the risk of neonatal morbidity among women with singleton pregnancies. Secondary analysis of data from the Maternal Fetal Medicine Units Network Factor V Leiden Mutation study. We categorized time of delivery as day (07:00-16:59), evening (17:00-23:59), and overnight (midnight-06:59). Severe neonatal morbidity was defined by at least one of the following: respiratory distress syndrome, transient tachypnea of the newborn, sepsis, seizures, neonatal intensive care admission, or a 5-minute APGAR ≤3. We calculated frequencies of severe neonatal morbidity by time of delivery. Multivariate analysis was performed to determine whether time of delivery was independently associated with severe neonatal morbidity. Among 4,087 women, 1,917 (46.9%) delivered during the day, 1,140 (27.9%) delivered in the evening, and 1,030 (25.2%) delivered overnight. We observed no significant differences in the rates of neonatal morbidity between delivery time periods (day: 12.3%; evening: 12.8%; overnight: 12.6%; p = 0.9). No significant association was observed between time of delivery and neonatal morbidity after adjustment for maternal, obstetric, and peripartum factors. Our findings suggest that time of delivery is not associated with severe neonatal morbidity. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  7. Death in CHARGE syndrome after the neonatal period

    NARCIS (Netherlands)

    Bergman, J. E. H.; Blake, K. D.; Bakker, M. K.; Sarvaas, G. J. du Marchie; Free, R. H.; van Ravenswaaij-Arts, C. M. A.

    CHARGE syndrome is a multiple congenital anomaly syndrome that can be life-threatening in the neonatal period. Complex heart defects, bilateral choanal atresia, esophageal atresia, severe T-cell deficiency, and brain anomalies can cause neonatal death. As little is known about the causes of death in

  8. Double-phase Tc-99m tetrofosmin parathyroid scan in hyperparathyroidism: comparison with ultrasonography

    Energy Technology Data Exchange (ETDEWEB)

    Kim, In Soo; Kim, Sang Yoon [Daegu Fatima Hospital, Daegu (Korea, Republic of); Zeon, Seok Kil; Won, Kyoung Sook [School of Medicine, Keimyung University, Daegu (Korea, Republic of)

    2004-07-01

    This study was performed to evaluate the utility of double-phase Tc-99m Tetrofosmin(TF) parathyroid scan in the detection of pathologic lesions of primary hyperparathyroidism, and comparison with the ultrasonography(US). The double phase TF parathyroid scan of the anterior neck including upper mediastinum with 800 MBq TF were acquired at ten minutes (early phase) and at two hours (delayed phase) after radiopharmaceutical injection, in 24 consecutive patients under the clinical impression of primary hyperparathyroidism and hypercalcaemia. The images were evaluated for abnormal focal areas of increased tracer localization in the anterior neck and superior mediastinum in early phase, and visualization of parathyroid gland radioactivity after wash-out of the thyroid gland radioactivity in delayed phase. US of the anterior neck including upper mediastinum was performed by a diagnostic radiologist in 24 consecutive patients, within one week before or after the scan. The findings of double phase TF parathyroid scan and US were compared with the pathologic results. Ten of 24 patients were surgically explored and pathologic results showed eight adenomas and two hyperplasia. The double phase TF parathyroid scan showed positive findings in seven patients of eight adenomas and one patient of two hyperplasia patients. US image showed positive findings in six patients of eight adenomas and no positive findings of two hyperplasia. The sensitivity of the double phase TF scan for detection of the causes of the primary hyperparathyroidism was 80% and US was 60%. The double phase Tc-99m Tetrofosmin parathyroid scan showed higher sensitivity in detection of the pathologic lesions of primary hyperparathyroidism than ultrasonography.

  9. Brown tumor of the patella caused by primary hyperparathyroidism: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Irie, Tomoko; Mawatari, Taro; Ikemura, Satoshi; Matsui, Gen; Iguchi, Takahiro; Mitsuyasu, Hiroaki [Orthopaedic Surgery, Hamanomachi Hospital, Fukuoka (Japan)

    2015-06-15

    It has been reported that the common sites of brown tumors are the jaw, pelvis, ribs, femurs and clavicles. We report our experience in a case of brown tumor of the patella caused by primary hyperparathyroidism. An initial radiograph and CT showed an osteolytic lesion and MR images showed a mixed solid and multiloculated cystic tumor in the right patella. One month after the parathyroidectomy, rapid bone formation was observed on both radiographs and CT images.1.

  10. Single Large Bladder Stone in a Young Male Adult with Primary Hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Omar Halalsheh

    2017-07-01

    Full Text Available Bladder stones are caused when minerals are built up in the bladder, especially if the bladder is incompletely emptied. These stones will pass while they are small. Otherwise, they get stuck to the bladder wall or ureter. If this happens, they gradually gather more mineral crystals, becoming larger over time. Primary hyperparathyroidism is usually caused by a tumor within the parathyroid gland, and elevated calcium levels can cause digestive symptoms, psychiatric abnormalities, bone disease and multiple kidney stones.

  11. Failure of thiazide diuretics to increase plasma calcium in mild primary hyperparathyroidism.

    OpenAIRE

    Farquhar, C. W.; Spathis, G. S.; J. L. Barron; Levin, G. E.

    1990-01-01

    Thirteen patients with mild primary hyperparathyroidism who were taking thiazide diuretics intermittently for periods of up to 18 months were followed up for a mean of 5.3 years. No significant difference was found in either plasma total calcium corrected for albumin or whole blood ionized calcium in these patients between the periods on or off thiazides. We conclude that thiazide diuretics are not contraindicated in such patients.

  12. Total parathyroidectomy combined with partial auto-transplantation for the treatment of secondary hyperparathyroidism

    Institute of Scientific and Technical Information of China (English)

    ZOU Qiang; ZHANG Yan-ling; WANG Hong-ying; ZHOU Jian; LAO Zheng-yin; XUE Jun; LI Ming-xin; LI Hai-ming; JIN Yi-ting; GU Yong

    2007-01-01

    Background Drug treatment for secondary hyperparathyroidism caused by chronic renal failure may be available at the early stage of the disease, but it is not as effective for serious patients. The aim of the study was to evaluate the effect of total parathyroidectomy combined with forearm autotransplantation in the uremic patients with secondary hyperparathyroidism.Methods From September 1999 through September 2006, parathroidectomy and autotransplantation was performed in 20 patients. The coherence between the results of preoperative parathyroid ultrasonography and surgical exploration were compared. The serum calcium concentration and intact parathyroid hormone (iPTH) were monitored preoperatively,intraoperatively, and postoperatively.Results A total of 71 hyperplastic parathyroid glands were resected in the 20 patients. The accordance rate of parathyroid localization between B-ultrasonography and intraoperative exploration was 94.4%. The average iPTH value was (110.90±67.42) ng/L, (433.80±243.72) ng/L, (48.80±42.69) ng/L, (229.04±172.68) ng/L and (232.39±224.05) ng/L at day 1, 2, 3, 7, 30 after operation respectively. The clinical symptoms were ameliorated and the levels of serum calcium concentration were controlled within the normal range after operation. Recurrent secondary hyperparathyroidism had happened in 1 case, 4 years postoperatively because of the development of autograft hyperplasia, and in another case 2years postoperatively due to remnant of neck parathyroid glands. The clinical symptoms were all alleviated after re-operation. No surgical complication had occurred in any of the patients.Conclusions The total parathyroidectomy with forearm autotransplantation is feasible, safe, and effective for patients with secondary hyperparathyroidism in the short term. The long-term effects should be further investigated.

  13. Spontaneous remission of primary hyperparathyroidism in a patient with neurofibromatosis type 1: case report

    OpenAIRE

    França, Mariana Martins; Santos, Antonio B. [UNESP; Hirosawa, Renata Midori [UNESP; Tagliarini, José Vicente [UNESP; Mazeto, Gláucia Maria Ferreira da Silva; Nunes, Vania Dos Santos [UNESP

    2012-01-01

    Neurofibromatosis type 1 (NF1) is an autosomal dominant multisystem disorder affecting approximately 1 in 3500 individuals. Patients with the disorder can develop carcinoid tumors, medullary thyroid carcinoma, pheochromocytoma and tumor of the hypothalamus. The association of NF1 with Primary Hyperparathyroidism (HPP) is very rare. We report a 56-year-old woman with NF1 who was referred to our service because of nephrolithiasis. Physical examination revealed the characteristic signs of NF1, a...

  14. Brown tumor of the patella caused by primary hyperparathyroidism: a case report.

    Science.gov (United States)

    Irie, Tomoko; Mawatari, Taro; Ikemura, Satoshi; Matsui, Gen; Iguchi, Takahiro; Mitsuyasu, Hiroaki

    2015-01-01

    It has been reported that the common sites of brown tumors are the jaw, pelvis, ribs, femurs and clavicles. We report our experience in a case of brown tumor of the patella caused by primary hyperparathyroidism. An initial radiograph and CT showed an osteolytic lesion and MR images showed a mixed solid and multiloculated cystic tumor in the right patella. One month after the parathyroidectomy, rapid bone formation was observed on both radiographs and CT images.

  15. Neonatal Outcomes of Rh-Negative Pregnancies in a Tertiary Level Neonatal Intensive Care Unit: A Prospective Study

    Directory of Open Access Journals (Sweden)

    Chacham

    2016-07-01

    Full Text Available Background Rhesus incompatibility is a preventable cause for severe neonatal hyperbilirubinemia, hydrops fetalis and still births. The prevalence of the Rh-negative blood group among Indian woman varies from 2% - 10%. Despite declining the incidence of Rhesus incompatibility, due to availability of anti-D immunoglobulin, and improved antenatal care of the Rh-negative pregnant woman, it still accounts for a significant proportion of neonatal hyperbilirubinemia and neuro-morbidity. The prevalence of Rh-negative women having Rh-positive neonates is 60%. Objectives This study aimed to estimate the incidence of Rh iso-immunization and evaluate the outcomes of Rh iso-immunized neonates. Methods This prospective observational study was conducted in a tertiary level neonatal intensive care unit, Princess Esra hospital, Deccan college of medical sciences, Hyderabad, Telangana, India. Consecutive intramural and extramural neonates admitted to neonatal intensive care unit with the Rh-negative mother’s blood group and hyperbilirubinemia were enrolled. Neonates born to Rh+ve mothers were excluded. Neonatal gestational age, birth weight, age at admission, duration of phototherapy, duration of hospitalization, neonatal examination and investigations were recorded in a predesigned, pretested performa. Results A total of 90 neonates were born to Rh-negative mothers, of which 70% (63 had the Rh-positive blood group and 30% had the Rh-negative blood group. Of these 63 neonates, 48 (76.2% had hyperbilirubinemia and 43 neonates (68.3% had significant hyperbilirubinemia (total serum bilirubin > 15mg/dL. Among them, 2%, 75% and 23% were born to primi, multi and grandmutli, respectively. Also, 14.5% of the neonates were large for dates (LFD, 75% appropriate for dates (AFD and 10.5% were small for dates (SFD. Premature and SFD neonates had higher incidence of hyperbilirubinemia. Significantly higher incidence of jaundice occurred within 72 hours of life. The mean

  16. Value of ultrasonography, Ct and MR imaging in the diagnosis of primary hyperparathyroidism

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    Tziakouri, C. [Nicosia General Hospital (Cyprus). Dept. of Radiology; Eracleous, E. [Nicosia General Hospital (Cyprus). Dept. of Radiology; Skannavis, S. [Nicosia General Hospital (Cyprus). Dept. of Radiology; Pierides, A. [Nicosia General Hospital (Cyprus). Dept. of Nephrology; Symeonides, P. [Nicosia General Hospital (Cyprus). Dept of General Surgery; Gourtsoyiannis, N. [Univ. of Crete, Heraklion (Greece). Dept. of Radiology

    1996-09-01

    Purpose: To evaluate the significance of preoperative localization of abnormal parathyroid glands to the surgical outcome in patients with primary hyperparathyroidism. Material and Methods: Thirty-nine patients with primary hyperparathyroidism were studied preoperatively with US (39 patients), CT (30 patients) and MR imaging (18 patients). The overall diagnostic accuracy for US was 87%, CT 66% and MR 94%. In patients with a single parathyroid adenoma US was the most cost-effective localization technique with a detection rate of 96%. CT had a lower detection rate (78%) but was of particular value for fairly large ectopic adenomas in the root of the neck. MR imaging was a good confirmatory test (93%). In patients with multiple gland disease (primary hyperplasia and multiple adenomas), no single localization study alone was sufficient. Combination of all 3 studies, however, alerted the physician to the presence of disease in more than one gland in 87% of these patients. Conclusion: US, CT and MR imaging followed by surgery performed by an experienced surgeon provided good clinical results in 39 patients with primary hyperparathyroidism. Preoperative localization was especially useful in patients with primary parathyroid hyperplasia or multiple adenomas and in patients with ectopic parathyroid adenomas in the root of the neck. We recommend identification of all abnormal parathyroid glands prior to surgery. (orig.).

  17. The role of ultrasound and nuclear medicine methods in the preoperative diagnostics of primary hyperparathyroidism

    Science.gov (United States)

    Cacko, Marek; Królicki, Leszek

    2015-01-01

    Primary hyperparathyroidism (PH) represents one of the most common endocrine diseases. In most cases, the disorder is caused by parathyroid adenomas. Bilateral neck exploration has been a widely used treatment method for adenomas since the 20's of the twentieth century. In the last decade, however, it has been increasingly replaced by a minimally invasive surgical treatment. Smaller extent, shorter duration and lower complication rate of such a procedure are emphasized. Its efficacy depends on a precise location of parathyroid tissue during the preoperative imaging. Scintigraphy and ultrasound play a major role in the diagnostic algorithms. The efficacy of both methods has been repeatedly verified and compared. The still-current guidelines of the European Association of Nuclear Medicine (2009) emphasize the complementary role of scintigraphy and ultrasonography in the preoperative diagnostics in patients with primary hyperparathyroidism. At the same time, attempts are made to improve both these techniques by implementing new study protocols or innovative technologies. Publications have emerged in the recent years in the field of ultrasonography, whose authors pointed out the usefulness of elastography and contrast media. Nuclear medicine studies, on the other hand, focus mainly on the assessment of new radiotracers used in the positron emission tomography (PET). The aim of this article is to present, based on literature data, the possibilities of ultrasound and scintigraphy in the preoperative diagnostics in patients with primary hyperparathyroidism. Furthermore, the main directions in the development of imaging techniques in PH patients were evaluated. PMID:26807297

  18. Surgical diagnosis and treatment of primary hyperparathyroidism: analysis of 19 cases.

    Science.gov (United States)

    Gao, Bo; Jiang, Yan; Zhang, Shu; Guo, Lingji; Tian, Wuguo; Wen, Yayuan; He, Yujun; Luo, Donglin

    2015-01-01

    This study was to discuss the surgical diagnosis and treatment experience of primary hyperparathyroidism. Clinical data of 19 primary hyperparathyroidism patients who were treated surgically in our department from Jan. 2005 to Jul. 2014 were retrospectively analyzed. Besides, general data, clinical manifestations, laboratory and imaging test results, surgical procedures and postoperative follow-up information were comprehensively analyzed. 15 of 19 patients had adenoma, among whom 1 case was complicated with goiter, 3 cases with parathyroid hyperplasia, and 1 case with parathyroid carcinoma. One case of bilateral parathyroid adenoma was explored bilaterally, and the bilateral parathyroid adenoma was excised. 14 cases of unilateral parathyroid adenoma were explored unilaterally and the unilateral parathyroid adenoma was excised. 3 cases of parathyroid hyperplasia were explored bilaterally, and parathyroid glands were removed subtotally, and only half gland was reserved. 1 case of parathyroid carcinoma experienced excision of thyroid gland and parathyroid at the affected side and isthmus excision, subtotal excision of thyroid gland at the healthy side and functional cervical lymphonode dissection at the affected side. All the 19 cases recovered well after operation, and symptoms of hyperparathyroidism were controlled. No relapse was found after follow-up of 3 months to 5 years. In conclusion, local parathyroid excision with small wounds after pre-operative locative image test and qualitative laboratory test is effective. Timely surgical treatment could reduce joint and urinary damage. Post-operative follow-up should be emphasized for early detection of the patients with hypoparathyroidism and recurrence.

  19. The localization of 10% of the pathologic glands in secondary hyperparathyroidism depends on the surgeon experience

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    José Luis D’Addino

    2015-07-01

    Full Text Available Methods for preoperative localization of parathyroidin secondary hyperparathyroidism are controversial in the literature and have different and dissimilar sensitivity. With the objective to determine the correlation between preoperative ultrasound, scintigraphy MIBI and intraoperative findings in secondary hyperparathyroidism we review our 10 years statistic.Between2004-2014, 100 patients underwent parathyroidectomy due to secondary hyperparathyroidism. Data obtained from medical records included: preoperative serum parathormona, ultrasound, scintigraphy. Positive predictive value and negative predictive value were analyzed in correlation with intraoperative findings.The method of calculation of ROC curves and area under the curve and other screening values (confidence index, index of validity and likelihood ratio were used. 68% were women; mean age was 52,7 years. Mean PTH value was 1486 pg/ml. The specificity and sensitivity of preoperative ultrasound were 94,44% and 30,14%, respectively. PPV was 93,62% and NPV was 33,33%. For scintigraphy, the sensitivity was 25,34%, specificity 98,15%, PPV was 97,37% and NPV was 32,72%. The ultrasound diagnosed 94 glands among a possibility of 400, the scintigraphy showed 76 and the surgery founded 292. Recurrence, 22%. Ultrasound and scintigraphy allow the localization of pathological parathyroid; however, in 10% of cases,glands could not be detected preoperatively, making surgeons experience fundamental in gland localization

  20. Hyperphosphatemia-induced hyperparathyroidism in 5/6 nephrectomized rats: development of a new animal model

    Institute of Scientific and Technical Information of China (English)

    JIANG Ying; WANG Mei

    2008-01-01

    Background We require a stable model to understand the molecular mechanism by which isolated hyperphosphatemia induces hyperparathyroidism secondary to chronic renal failure.The present study established a rat model of hyperphosphatemia-induced secondary hyperparathyroidism in chronic renal failure.Methods Twenty-nine rats with 5/6 nephrectomy (Nx) were divided into three groups and were fed for 10 weeks on a high phosphate diet (1.2% phosphate) starting from three different post-Nx time points.Parathyroid hormone mRNA in parathyroid gland was measured by real-time PCR and parathyroid cell hyperplasia was tested by proliferating cell nuclear antigen (PCNA) assay.Results The 10 rats fed a high phosphate diet starting from the fourth week post-Nx had isolated hyperphosphatemia and excess synthesis/secretion of parathyroid hormone,and hyperplasia of the parathyroid glands were induced (r=0.86-0.97,P<0.001),but the levels of serum calcium and 1,25(OH)2D3 did not change.Conclusion A rat model of hyperphosphatemia-induced secondary hyperparathyroidism in chronic renal failure was established by 5/6 Nx and 10 weeks-high phosphate diet starting from the fourth week post-Nx.

  1. Can SPECT change the surgical strategy in patients with primary hyperparathyroidism?

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    Iervolino, Leticia; Scalisse, Nilza Maria; Maeda, Sergio Setsuo, E-mail: leiervolino@hotmail.com [Discipline of Endocrinology, Department of Medicine, Faculdade de Medicina da Santa Casa de Sao Paulo (FMSCSP), Sao Paulo, SP (Brazil)

    2012-06-15

    Primary hyperparathyroidism (PHPT) is the most common cause of hypercalcaemia in outpatients. It is more common in females, after menopause, and the prevalence is 1 to 4:1000 in the general population. Patients with PHPT have abnormal regulation of PTH secretion, resulting in elevated serum calcium and inappropriately high or normal PTH in relation to the calcium value. Sporadic PTH-secreting adenoma alone accounts for 90% of cases of PHPT, while multiglandular hyperplasia is more common in familial hyperparathyroidism syndromes (5%) and parathyroid carcinomas represent less than 1% of cases. Only after making sure there is functional autonomy of one or more parathyroid glands, localization imaging tests should be performed to guide a possible surgical procedure. It is important to highlight that these tests have limitations and can yield false-positive and false-negative results. There are cases in which the parathyroid gland is difficult to be located, requiring a combination of imaging methods for pre-operative localization, such as {sup 99m}Tc-pertechnetate, SPECT, SPECT/CT, and US. We describe the case of a 50-year-old female patient diagnosed with PHPT, who underwent a surgical procedure without success, with maintenance of hypercalcaemia and hyperparathyroidism. In this case, the hyper functioning parathyroid was located in the retrotracheal region only after scintigraphy combined with SPECT/CT were used. (author)

  2. A systematic review of the diagnosis and treatment of primary hyperparathyroidism from 1995 to 2003.

    Science.gov (United States)

    Ruda, James M; Hollenbeak, Christopher S; Stack, Brendan C

    2005-03-01

    To systematically review the current preoperative diagnostic modalities, surgical treatments, and glandular pathologies associated with primary hyperparathyroidism. A systematic literature review. Of the 20,225 cases of primary hyperparathyroidism reported, solitary adenomas (SA), multiple gland hyperplasia disease (MGHD), double adenomas (DA), and parathyroid carcinomas (CAR) occurred in 88.90%, 5.74%, 4.14%, and 0.74% of cases respectively. Tc 99m -sestamibi and ultrasound were 88.44% and 78.55% sensitive, respectively, for SA, 44.46% and 34.86% for MGHD, and 29.95% and 16.20% for DA, respectively. Postoperative normocalcemia was achieved in 96.66%, 95.25%, and 97.69% of patients offered minimally invasive radio-guided parathyroidectomy (MIRP), unilateral, and bilateral neck exploration (BNE). Intraoperative PTH assays (IOPTH) were helpful in approximately 60% of bilateral neck exploration conversion (BNEC) surgeries. The overall prevalence of multiple gland disease (MGD and DA) was lower than often suggested by conventional wisdom. Furthermore, preoperative imaging was less accurate than it is often perceived for accurately imaging MGD. MIRP and UNE were more successful in achieving normocalcemia than is typically quoted. IOPTH was a helpful but not "fool-proof" adjunct in parathyroid exploration surgery. These results support a greater role for the treatment of primary hyperparathyroidism using less invasive approaches. EMB rating: B-3.

  3. [Usefulness of computed tomography and magnetic resonance in the preoperative diagnosis for hyperparathyroidism].

    Science.gov (United States)

    Pino Rivero, V; Pantoja Hernández, C G; González Palomino, A; Trinidad Ruíz, G; Marcos García, M; Keituqwa Yáñez, T; Pardo Romero, G; Blasco Huelva, A

    2005-01-01

    Sonnography and Tc-99m sestamibi scintigraphy are the most requested preoperative imaging tests nowdays in the surgery of hyperparathyroidism. The aim of our article is to know if Computerized Tomography (CT) and Magnetic Resonance Imaging (MRI) are useful as a location study and in which cases it would be more justified to ask these radiologic techniques. For that we report our results with 29 patients at all diagnosed as hyperparathyroidism (26 primary forms and 3 secondary ones) and operated by our E.N.T. Department later. On 20 of them a cervical CT was asked before the parathyroidectomy and on the rest 9, a MRI with sensitivities of 65% and 88.9% respectively. We think both complementary explorations must not be solicited by routine but they can represent a help in the cases in that sonnography and scintigraphy are not able to show the possible adenoma or hiperplasia, particularly in recurrent hyperparathyroidisms, reinterventions or suspect of parathyroid glands in an atypical location.

  4. Relative hyperphosphaturia in diabetic chronic renal failure: a protective factor of hyperparathyroidism.

    Science.gov (United States)

    Aubia, J; Bosch, J; Lloveras, J; Chine, M; Hojman, L; Masramon, J

    1987-01-01

    Relative low serum levels of parathormone (PTH) and low incidence of secondary hyperparathyroidism have been reported in diabetic uremic patients. The pathogenesis of this reported resistance to uremic secondary hyperparathyroidism in diabetes remains controversial. We have measured the serum C-terminal parathormone (C-PTH) renal phosphorus threshold (TmPO4) and nephrogenous cyclic AMP (N-cCAMP), in 2-hour urine collection in 22 patients with diabetic nephropathy with moderate chronic renal failure and in 27 controls with similar creatinine clearance values (18.16 +/- 9.14 and and 19.1 +/- 8.47 ml/min). In spite of the lower levels of serum C-PTH (1.07 +/- 0.43 ng/ml) diabetic patients exhibited an increased phosphaturia (TmPO4: 1.97 +/- 0.9 mg/100 ml GFR) when compared with the control group (C-PTH: 2.01 +/- 1.17 mg/ml, and TmPO4: 2.5 +/- 0.7 ml GFR). When the C-PTH values were plotted against the logarithm of creatinine clearance values, both groups showed a significant linear relationship reflecting the progressive increase in PTH when GFR fell. This progressive parathyroid stimulus was also present in diabetic patients but in a lower intensity. We believe that increased phosphaturia in diabetics with moderate chronic renal failure may be a major factor in precluding the appearance of secondary hyperparathyroidism in these patients once they reach the dialysis and transplantation programs.

  5. Neonatal asphyxia and forensic medicine.

    Science.gov (United States)

    d'Aloja, E; Müller, M; Paribello, F; Demontis, R; Faa, A

    2009-01-01

    In the last decades, the scientific literature addressing neonatal encephalopathy has grown in a logarithmic way and malpractice claims in obstetrics and neonatology have become a major threat to the health service. At the moment, scientific evidence are insufficient to clearly identify in each single case whether the hypoxic insult has developed in the course of labor or in the first few hours after the birth or, otherwise, whether the damage has to recognize a remote and long-lasting cause acting during pregnancy. Several authors feel that this scientific uncertainty leads to a higher percentage of civil suit decisions prone to recognizing a guilty medical behavior, and they wish a more in-depth analysis of all these cases to clearly identify all the data either in favor or in contrary to the assumption of the existence of a causal correlation between neonatal encephalopathy and medical misbehavior. This article will focus on the medico-legal approach to a hypoxic-ischemic event in the perinatal period, addressing the relevant data to be collected in order to establish the medical and juridical cause of the neonatal damage.

  6. The neonatal chest

    Energy Technology Data Exchange (ETDEWEB)

    Lobo, Luisa [Servico de Imagiologia Geral do Hospital de Santa Maria, Av. Prof. Egas Moniz, 1649-035 Lisbon (Portugal)]. E-mail: mluisalobo@gmail.com

    2006-11-15

    Lung diseases represent one of the most life threatening conditions in the newborn. Important progresses in modern perinatal care has resulted in a significantly improved survival and decreased morbidity, in both term and preterm infants. Most of these improvements are directly related to the better management of neonatal lung conditions, and infants of very low gestational ages are now surviving. This article reviews the common spectrum of diseases of the neonatal lung, including medical and surgical conditions, with emphasis to the radiological contribution in the evaluation and management of these infants. Imaging evaluation of the neonatal chest, including the assessment of catheters, lines and tubes are presented.

  7. Risk factors for hearing loss in neonates

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    Ni Luh Putu Maharani

    2016-11-01

    Full Text Available Background An estimated 6 of 1,000 children with live birthssuffer from permanent hearing loss at birth or the neonatal period.At least 90% of cases occur in developing countries. Hearing lossshould be diagnosed as early as possible so that intervention canbe done before the age of 6 months.Objective To determine risk factors for hearing loss inneonates.Methods We performed a case-control study involving 100neonates with and without hearing loss who were born atSanglah Hospital, Denpasar from November 2012 to February2013. Subjects were consisted of 2 groups, those with hearingloss (case group of 50 subjects and without hearing loss (controlgroup of 50 subjects. The groups were matched for gender andbirth weight. We assessed the following risk factors for hearingloss: severe neonatal asphyxia, hyperbilirubinemia, meningitis,history of aminoglycoside therapy, and mechanical ventilationby Chi-square analysis. The results were presented as odds ratioand its corresponding 95% confidence intervals.Results Seventy percent of neonates with hearing loss had historyof aminoglycoside therapy. Multivariable analysis revealed thataminoglycoside therapy of 14 days or more was a significant riskfactor for hearing loss (OR 2.7; 95%CI 1.1 to 6.8; P=0.040.There were no statistically significant associations betweenhearing loss and severe asphyxia, hyperbilirubinemia, meningitis,or mechanical ventilation.Conclusion as a risk factor for hearing loss in neonates. [

  8. [Seven cases of parathyroidectomy for secondary hyperparathyroidism using methylene blue: suggestion for the method of methylene blue infusion].

    Science.gov (United States)

    Kadoya, Tatsuo; Kinoshita, Yuki; Shiraishi, Munehiro; Uehara, Hirofumi; Yamamoto, Toshinori; Suetsugu, Keiko

    2014-08-01

    Intraoperative staining of the parathyroid glands with intravenously administered methylene blue is well described and has been demonstrated as an effective and safe method to facilitate parathyroidectomy. However, there have been several literatures of the development of postoperative neurological toxicity in patients who received methylene blue infusion during parathyroidectomy. We report the method of methylene blue infusion during parathyroidectomy at our institution. Seven adult patients who had undergone parathyroidectomy for secondary hyperparathyroidism associated with chronic renal failure were included in this study. Methylene blue was administered at a constant rate of 4 mg x kg(-1) x hr(-1) with a 1% solution just before the start of operation. The infusion was stopped after the first parathyroid gland was identified. The mean dose of methylene blue used was 2.2 +/- 0.8 mg x kg(-1). Consequently, the dose of methylene blue by this method could be decreased to less than half of the previously administered dose (6 mg x kg(-1)) at our institution. The dose of methylene blue used should be kept to the minimum required to identify the parathyroid glands in each case.

  9. Malignant insulinoma with hepatic and pulmonary metastases associated with primary hyperparathyroidism. Case report and review of the literature.

    Science.gov (United States)

    Albu, A; Zirnea, A; Georgescu, O; Terzea, D; Jinga, D; Fica, S

    2008-01-01

    Malignant insulinomas are rare tumors (10% of insulinomas) that often present as mnulticentric macro nodules with multiple liver metastases before diagnosis. We report the case of a 55 year old female with a medical history of severe hypoglycemic attacks for two months. Blood tests showed a decreased value of glycemia (30 mg/dl) associated with increased insulin level (l6 microU/ml) and an increased glycemia/insulinemia ratio of 1.87 supporting the diagnosis of insulinoma. Abdominal CT showed a 1.5 cm mass localized in the head of the pancreas with disseminated hepatic tumors, confirmed as neuroendocrine metastases by biopsy (which proved the presence of a malignant insulinoma). Primary hyperparathyroidism was diagnosed based on mild elevation of calcium (10.4 mg/dl) associated with a high level of PTH (71.2 pg/ml). The coexistence of the two endocrinopathies suggested the presence of type 1 multiple endocrine neoplasia (MEN 1). Because of multiple hepatic masses and liver function impairment, surgery and hepatic artery embolization were not performed. Somatostatin analog therapy was started with symptomatic control in the beginning, but rapid loss of beneficial effect. Finally, systemic chemotherapy with doxorubicin was administered, but the disease was progressive and after three months we decided to stop it. The patient died at home after one month, probably in hypoglycemic coma.

  10. Neonatal pain management

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    Tarun Bhalla

    2014-01-01

    Full Text Available The past 2-3 decades have seen dramatic changes in the approach to pain management in the neonate. These practices started with refuting previously held misconceptions regarding nociception in preterm infants. Although neonates were initially thought to have limited response to painful stimuli, it was demonstrated that the developmental immaturity of the central nervous system makes the neonate more likely to feel pain. It was further demonstrated that untreated pain can have long-lasting physiologic and neurodevelopmental consequences. These concerns have resulted in a significant emphasis on improving and optimizing the techniques of analgesia for neonates and infants. The following article will review techniques for pain assessment, prevention, and treatment in this population with a specific focus on acute pain related to medical and surgical conditions.

  11. Sonomammography in Neonatal Mastauxe

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    Sushil Ghanshyam Kachewar

    2015-03-01

    Full Text Available Prominence or even enlargement of one or both breasts is known in neonates. It is believed to be a physiological response to falling levels of maternal estrogen towards last trimester of pregnancy. This input stimulates prolactin release from the newborn's pituitary leading to transient neonatal breast enlargement. This phenomenon is independent of the gender of the neonate. It presents in the first few weeks of life and resolves subsequently. Often fluid discharge is noted from the prominent or swollen breast that resolves without treatment in subsequent weeks. Manual breast manipulation for discharge removal may lead to undesirable effects like local irritation, enhanced enlargement, prolonged tissue hypertropy or even mastitis. A case of such 7-days female neonate is presented here backed with imaging evaluation for confirmation of diagnosis. Typical sonomammographic findings are described. [Cukurova Med J 2015; 40(Suppl 1: 22-24

  12. Maternal and neonatal tetanus

    Science.gov (United States)

    Thwaites, C Louise; Beeching, Nicholas J; Newton, Charles R

    2017-01-01

    Maternal and neonatal tetanus is still a substantial but preventable cause of mortality in many developing countries. Case fatality from these diseases remains high and treatment is limited by scarcity of resources and effective drug treatments. The Maternal and Neonatal Tetanus Elimination Initiative, launched by WHO and its partners, has made substantial progress in eliminating maternal and neonatal tetanus. Sustained emphasis on improvement of vaccination coverage, birth hygiene, and surveillance, with specific approaches in high-risk areas, has meant that the incidence of the disease continues to fall. Despite this progress, an estimated 58 000 neonates and an unknown number of mothers die every year from tetanus. As of June, 2014, 24 countries are still to eliminate the disease. Maintenance of elimination needs ongoing vaccination programmes and improved public health infrastructure. PMID:25149223

  13. Correction of Neonatal Hypovolemia

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    V. V. Moskalev

    2007-01-01

    Full Text Available Objective: to evaluate the efficiency of hydroxyethyl starch solution (6% refortane, Berlin-Chemie versus fresh frozen plasma used to correct neonatal hypovolemia.Materials and methods. In 12 neonatal infants with hypoco-agulation, hypovolemia was corrected with fresh frozen plasma (10 ml/kg body weight. In 13 neonates, it was corrected with 6% refortane infusion in a dose of 10 ml/kg. Doppler echocardiography was used to study central hemodynamic parameters and Doppler study was employed to examine regional blood flow in the anterior cerebral and renal arteries.Results. Infusion of 6% refortane and fresh frozen plasma at a rate of 10 ml/hour during an hour was found to normalize the parameters of central hemodynamics and regional blood flow.Conclusion. Comparative analysis of the findings suggests that 6% refortane is the drug of choice in correcting neonatal hypovolemia. Fresh frozen plasma should be infused in hemostatic disorders. 

  14. Neonatal Herpes Simplex Virus Infection.

    Science.gov (United States)

    James, Scott H; Kimberlin, David W

    2015-09-01

    Herpes simplex virus (HSV) 1 and HSV-2 infections are highly prevalent worldwide and are characterized by establishing lifelong infection with periods of latency interspersed with periodic episodes of reactivation. Acquisition of HSV by an infant during the peripartum or postpartum period results in neonatal HSV disease, a rare but significant infection that can be associated with severe morbidity and mortality, especially if there is dissemination or central nervous system involvement. Diagnostic and therapeutic advances have led to improvements in mortality and, to a lesser extent, neurodevelopmental outcomes, but room exists for further improvement. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Neonatal renal vein thrombosis.

    Science.gov (United States)

    Brandão, Leonardo R; Simpson, Ewurabena A; Lau, Keith K

    2011-12-01

    Neonatal renal vein thrombosis (RVT) continues to pose significant challenges for pediatric hematologists and nephrologists. The precise mechanism for the onset and propagation of renal thrombosis within the neonatal population is unclear, but there is suggestion that acquired and/or inherited thrombophilia traits may increase the risk for renal thromboembolic disease during the newborn period. This review summarizes the most recent studies of neonatal RVT, examining its most common features, the prevalence of acquired and inherited prothrombotic risk factors among these patients, and evaluates their short and long term renal and thrombotic outcomes as they may relate to these risk factors. Although there is some consensus regarding the management of neonatal RVT, the most recent antithrombotic therapy guidelines for the management of childhood thrombosis do not provide a risk-based algorithm for the acute management of RVT among newborns with hereditary prothrombotic disorders. Whereas neonatal RVT is not a condition associated with a high mortality rate, it is associated with significant morbidity due to renal impairment. Recent evidence to evaluate the effects of heparin-based anticoagulation and thrombolytic therapy on the long term renal function of these patients has yielded conflicting results. Long term cohort studies and randomized trials may be helpful to clarify the impact of acute versus prolonged antithrombotic therapy for reducing the morbidity that is associated with neonatal RVT.

  16. Pattern and prevalence of neonatal thrombocytopenia in Port Harcourt, Nigeria

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    Zaccheaus A Jeremiah

    2010-04-01

    Full Text Available Zaccheaus A Jeremiah1, Justina E Oburu21Hematology and Blood Transfusion Science Unit, Department of Medical Laboratory Sciences, College of Health Sciences, Niger Delta University, Wilberforce Island, Bayelsa State, Nigeria; 2Department of Hematology and Blood Transfusion University of Port Harcourt Teaching Hospital, Port Harcourt, NigeriaBackground: In Port Harcourt, evidence-based guidelines for platelet transfusion therapy in neonatal patients are yet to be defined and the prevalence and pattern of neonatal thrombocytopenia has not yet been reported.Methods: Platelet counts of 132 neonatal patients admitted into the special care baby unit (SCBU at the University of Port Harcourt Teaching Hospital in Nigeria were assessed using the International Committee on Standards in Hematology (ICSH approved manual procedures for hemocytometry.6Study design: This is a cross sectional study carried out on neonates to determine the prevalence and pattern of neonatal thrombocytopenia.Results: The median platelet count of the neonates was 97.0 × 109/L (interquartile range [IQR] 50–152 while the mean age was 61.7 hours (range 1–336 hours. The overall prevalence of neonatal thrombocytopenia was 53.0%. Mild thrombocytopenia (platelet count 51–100 × 109/L was found in 39.4% of the neonates, 12.1% had moderate thrombocytopenia (platelet count 30–50 × 109/L, while severe thrombocytopenia (platelet count <30 × 109/L was detected in 1.5% of the neonates. Of these, 84.84% of the cases occurred within 72 hours (early onset. The most common clinical diagnosis among the neonates was severe birth asphyxia (33.3%, followed by neonatal jaundice (19.7%, neonatal sepsis (16.7%, low birth weight (13.6%, anemia and bleeding (6.1%, and other clinical conditions (10.6%. There was no association between clinical diagnosis and thrombocytopenia (Fisher’s exact test = 10.643; P = 0.923.Conclusion: There is a high prevalence of early onset neonatal thrombocytopenia

  17. RELEASE OF TONGUE-TIE IN NEONATES

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    V. Raveenthiran

    2012-01-01

    Full Text Available Arguably, release of tongue tie is the oldest surgical procedure and it is perhaps older than circumcision. According to the Old Testament, the Lord released the tied tongue of Moses and made him speak well. Tongue-tie is pedantically known as ankyloglossia inferior minor. Simplicity of its treatment has earned this malady several etiological attributions such as difficulty of speech and breast feeding. All over the world, lactation consultants refer neonates for release of tongue tie as they believe it to be the cause of maternal nipple pain.Perhaps this is the only indication of tongue tie release in neonates.

  18. Neonatal capsaicin causes compensatory adjustments to energy homeostasis in rats

    NARCIS (Netherlands)

    van de Wall, E. H. E. M.; Wielinga, P. Y.; Strubbe, J. H.; van Dijk, G.

    2006-01-01

    Several mechanisms involved in ingestive behavior and neuroendocrine activity rely on vagal afferent neuronal signaling. Seemingly contradictory to this idea are observations that vagal afferent neuronal ablation by neonatal capsaicin (CAP) treatment has relatively small effects on glucose homeostas

  19. Neonatal management and outcome in red cell alloimmunization

    NARCIS (Netherlands)

    Smits-Wintjens, Vivianne Elise Huberta Johanna

    2012-01-01

    In this thesis, several studies on neonatal red cell alloimmune hemolytic disease are presented, including various management options, associated complications and co-morbidities and the short-term and long-term outcome of children with Rhesus hemolytic disease.

  20. Neonatal capsaicin causes compensatory adjustments to energy homeostasis in rats

    NARCIS (Netherlands)

    van de Wall, E. H. E. M.; Wielinga, P. Y.; Strubbe, J. H.; van Dijk, G.

    2006-01-01

    Several mechanisms involved in ingestive behavior and neuroendocrine activity rely on vagal afferent neuronal signaling. Seemingly contradictory to this idea are observations that vagal afferent neuronal ablation by neonatal capsaicin (CAP) treatment has relatively small effects on glucose homeostas

  1. Neonatal management and outcome in red cell alloimmunization

    NARCIS (Netherlands)

    Smits-Wintjens, Vivianne Elise Huberta Johanna

    2012-01-01

    In this thesis, several studies on neonatal red cell alloimmune hemolytic disease are presented, including various management options, associated complications and co-morbidities and the short-term and long-term outcome of children with Rhesus hemolytic disease.

  2. Non-psychogenic polydipsia in 45-year-old man with primary hyperparathyroidism and recurrent bilateral nephrolithiasis

    Directory of Open Access Journals (Sweden)

    Rahmat Cahyanur

    2012-11-01

    Full Text Available Non-psychogenic polydipsia with hyponatremia is a rare clinical presentation. Primary hyperparathyroidism is a disorder of calcium, phosphate, and bone metabolism caused by increased level of parathyroid hormone (PTH. It is estimated the incidence of primary hyperparathyroidism are 21.6 per 100,000 person a year. This case report describe a 45-year-old man presented with non-psychogenic polydipsia. This patient drank a lot of water out of the fear of recurrent kidney stones. He had history of recurrent nephrolithiasis with hypercalcemia. We investigate further the cause of hypercalcemia and we diagnosed primary hyperparathryoidism as the cause. (Med J Indones. 2012;21:230-4Keywords: Hyponatremia, non-psychogenic polydipsia, primary hyperparathyroidism

  3. [Invasive candidiasis in neonatal intensive care units].

    Science.gov (United States)

    Brissaud, O; Tandonnet, O; Guichoux, J

    2011-05-01

    In the USA, the incidence of invasive candidiasis in neonates is respectively 0.3% of infants over 2500 g and up to 20% of infants less than 1000 g. Their incidence is increasing. Two populations of newborn infants are particularly vulnerable: the premature infants and newborn infants with severe neonatal digestive diseases. Fifty percent of infants hospitalized in NICU are colonized with Candida at the end of the first week of hospitalization; a direct relationship exists between the importance of colonization and the invasive infection risk. C. albicans is the species most often responsible for invasive candidiasis in the newborn. These infections represent the third cause of related-catheter infection in the USA. Mortality rate in neonates linked to this disease is 20 to 50%; morbidity primarily concerns brain and lungs. Neonatal invasive candidiasis risk factors are known and a primary prevention is possible. The diagnosis of neonatal invasive candidiasis is difficult and often delayed because of a polymorphic clinical expression. Empiric and preemptive treatment are based on the use of amphotericin B. Prophylactic treatment using fluconazole of newborns with birth weight ≤ 1000 grams and/or gestational age ≤ 27 weeks gestation is recommended by the American Academy of Paediatrics and the Infectious Diseases Society of America. A better knowledge of French epidemiological data in this area would improve both the diagnosis and therapeutic management of this disease.

  4. Cytomegalovirus in pregnancy and the neonate

    Science.gov (United States)

    Emery, Vincent C.; Lazzarotto, Tiziana

    2017-01-01

    Congenital cytomegalovirus (CMV) remains a leading cause of disability in children. Understanding the pathogenesis of infection from the mother via the placenta to the neonate is crucial if we are to produce new interventions and provide supportive mechanisms to improve the outcome of congenitally infected children. In recent years, some major goals have been achieved, including the diagnosis of primary maternal CMV infection in pregnant women by using the anti-CMV IgG avidity test and the diagnosis and prognosis of foetal CMV infection by using polymerase chain reaction real-time tests to detect and quantify the virus in amniotic fluid. This review summarises recent advances in our understanding and highlights where challenges remain, especially in vaccine development and anti-viral therapy of the pregnant woman and the neonate. Currently, no therapeutic options during pregnancy are available except those undergoing clinical trials, whereas valganciclovir treatment is recommended for congenitally infected neonates with moderately to severely symptomatic disease. PMID:28299191

  5. 高龄孕妇早发型重度先兆子痫对新生儿及围生结局影响分析%THE EFFECT OF EARLY ONSET SEVERE PRE- ECLAMPSIA OF ADVANCED AGE PREGNANT WOMEN ON NEONATAL AND PERINATAL OUTCOMES

    Institute of Scientific and Technical Information of China (English)

    胡水珍; 叶春波

    2011-01-01

    目的 分析高龄孕妇早发型重度先兆子痫对新生儿及围生结局的影响.方法 回顾性分析1997年1月-2010年1月早发型重度先兆子痫孕妇86例,其中年龄>35岁40例,设为研究组,年龄0.05).终止妊娠时间及孕妇严重并发症,差异有统计学意义(P<0.01).研究组围生儿病死率及颅内出血,新生儿窒息明显高于对照组(P<0.01).研究组新生儿出生体质量及新生儿Apgar评分明显小于对照组(P<0.01).结论 年龄>35岁孕妇发生早发性重度子痫,更易导致孕妇及围生儿预后不良.%Objective To analyze the effect of early onset severe pre - eclampsia( EOSP ) of advanced age pregnant women on neonatal and perinatal outcomes. Methods Eighty - six pregnant women with EOSP from Jan 1997 to Jan 2010 were chosen and analysed. According to the maternal age, 86 pregnant women with EOSP were classified into two groups, research group( the maternal age was up to 35 years and 40 patients were involved ) and control group( the maternal age was under 35 years and 46 patients were involved ). The parity, gestational age on admission, systolic pressure( SP ) and diastolic pressure( DP ) were recorded. And the pregnant outcomes of gravidas ( such as placental abruption, pneumonedema, heart failure ) and complications of perineal infants( such as perinatal mortality ratio, low birth weight, intracranial hemorrhage, neonatal asphyxia, Apgar score ) were recorded and compared between two groups. Results The parity, gestational age on admission, SP and DP had no different between two groups( P > 0.05 ). The duration of pregnancy termination and severe complication were different between two groups (P < 0. 01 ). Compared to control group, the perinatal mortality ratio, intracranial hemorrhage and neonatal asphyxia in research group were higher ( P < 0.01 ), and the birth weight, Apgar score in research group were lower ( P < 0.01 ). Conclusion The EOSP of advanced age pregnant women could

  6. Cinacalcet therapy in patients affected by primary hyperparathyroidism associated to Multiple Endocrine Neoplasia Syndrome type 1 (MEN1).

    Science.gov (United States)

    Giusti, Francesca; Cianferotti, Luisella; Gronchi, Giorgio; Cioppi, Federica; Masi, Laura; Faggiano, Antongiulio; Colao, Annamaria; Ferolla, Piero; Brandi, Maria Luisa

    2016-06-01

    Primary hyperparathyroidism is the main endocrinopathy associated with Multiple Endocrine Neoplasia type 1 syndrome. Cinacalcet is a calcimimetic agent licensed for the treatment of secondary hyperparathyroidism in patients with end-stage renal disease, and for the reduction of marked hypercalcemia in patients with parathyroid carcinoma and sporadic hyperparathyroidism requiring surgery but for whom parathyroidectomy is contraindicated. It may provide a medical alternative for the management of primary hyperparathyroidism in subjects affected by Multiple Endocrine Neoplasia type 1. In this longitudinal, intervention study, 33 MEN1 patients had been enrolled, 10 males and 23 females with a mean age of 40 ± 11.9 years, range 20-63. Primary hyperparathyroidism was the first clinical manifestation in 12 patients. All subjects commenced with Cinacalcet 30 mg/day, 22 patients starting therapy with calcimimetics as an alternative to surgery, and 11 patients opting for the medication after the onset of persistent post-surgical primary hyperparathyroidism. Duration of follow-up was 12 months. The results of this study show significant reductions in serum calcium. The changes in hormonal secretions of pituitary and gastroenteropancreatic glands were not significant, demonstrating the overall safety of this drug in this disease. Cinacalcet has been well tolerated by 28 patients, whereas five individuals complained of heartburn and grade 1 nausea, which did not prevent the completion of the study. In conclusion, Cinacalcet has resulted to be well tolerated and safe in patients with MEN1 syndrome and the calcium homeostasis was stabilized.

  7. Low dose four-dimensional computerized tomography with volume rendering reconstruction for primary hyperparathyroidism: How I do it?

    Institute of Scientific and Technical Information of China (English)

    Timothy; A; Platz; Moshim; Kukar; Rania; Elmarzouky; William; Cance; Ahmed; Abdelhalim

    2014-01-01

    Abstract Modification of 4-dimensional computed tomography(4D-CT)technique with volume rendering reconstruc-tions and significant dose reduction is a safe and ac-curate method of pre-operative localization for primary hyperparathyroidism.Modified low dose 4D-CT with volume rendering reconstructions provides precise preoperative localization and is associated with a sig-nificant reduction in radiation exposure compared to classic preoperative localizing techniques.It should be considered the preoperative localization study of choice for primary hyperparathyroidism.

  8. Primary Hyperparathyroidism. Statistical Study about Patient to bed in Imam Khomeiny and Shariaty Hospitals (67-1376 Years

    Directory of Open Access Journals (Sweden)

    Kafie N

    1998-09-01

    Full Text Available In this study we try to clear the prevalance of age and sex and clinical and radiographic"nmanifestations of primary hyperparathyroidism. In this duration 47 patients with primary"nhyperparathyroidism that bed in two important hospitals in Tehran were studied."nThe ratio between women to men was 7 to 1 and most prevalance of disease were in fouyth decade. The pain boon was the cheif complain of patients and osteopenia was the most radiographic findings."nIn addition a case of primary hyperparathyroidism with mandibular and other bone involvement was reported.

  9. Primary Hyperparathyroidism in Young People. When Should We Perform Genetic Testing for Multiple Endocrine Neoplasia 1 (MEN-1)?

    DEFF Research Database (Denmark)

    Lassen, Tina Harmer; Friis-Hansen, Lennart Jan; Rasmussen, Åse Krogh

    2014-01-01

    CONTEXT: Multiple endocrine neoplasia (MEN-1) is a rare, autosomal dominant inherited disorder. Primary hyperparathyroidism (pHPT) is the most frequent and usually the earliest expression of MEN-1, with typical age of onset at 20-25 years. Early detection of the disease and correct treatment...... endocrine neoplasia 1 OR multiple endocrine neoplasia type 1 AND Mutational analysis OR genetic testing OR testing OR Hyperparathyroidism, primary [majr]. A total of 625 abstracts were reviewed. RESULTS AND DISCUSSION: Whether to perform screening of patients with pHPT under the age of 30, 35, or 40 years...

  10. Treatment of secondary hyperparathyroidism in kidney disease: what we know and do not know about use of calcimimetics and vitamin D analogs

    Directory of Open Access Journals (Sweden)

    James B Wetmore

    2008-11-01

    Full Text Available James B Wetmore1, L Darryl Quarles1,21Department of Medicine, Division of Nephrology, University of Kansas Medical Center, Kansas City, KS, USA; 2The Kidney Institute, University of Kansas Medical Center, Kansas City, KS, USAAbstract: There is a growing understanding of the pathophysiology of secondary hyperparathyroidism (SHPT and a recent emergence of new agents for SHPT treatment in patients with advanced kidney disease. At the same time, appreciation that mineral metabolic derangements promote vascular calcification and contribute to excess mortality, along with recognition of potentially important “non-classical” actions of vitamin D, have prompted the nephrology community to reexamine the use of various SHPT treatments, such as activated vitamin D sterols, phosphate binders, and calcimimetics. In this review, the evidence for treatment of SHPT with calcimimetics and vitamin D analogs is evaluated, with particular consideration given to recent clinical trials that have reported encouraging findings with cinacalcet use. Additionally, several controversies in the pathogenesis and treatment of SHPT are explored. The proposition that calcitriol deficiency is a true pathological state is challenged, the relative importance of the vitamin D receptor and the calcium sensing receptor in parathyroid gland function is summarized, and the potential relevance of non-classical actions of vitamin D for patients with advanced renal disease is examined. Taken collectively, the balance of evidence now supports a treatment paradigm in which calcimimetics are the most appropriate primary treatment for SHPT in the majority of end stage renal disease patients, but which nevertheless acknowledges an important role for modest doses of activated vitamin D sterols.Keywords: secondary hyperparathyroidism, vitamin D, vitamin D receptor, calcium sensing receptor, calcimimetics, kidney disease

  11. Lesion localization in patients with hyperparathyroidism using double-phase Tc-99m MIBI parathyroid scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Jung Woo; Ryu, Jin Sook; Kim, Jae Seung; Moon, Dae Hyuk; Hong, Seung Mo; Gong, Gyung Yub; Hong, Suk Joon; Lee, Hee Kyung [College of Medicine, Ulsan Univ., Seoul (Korea, Republic of)

    1999-02-01

    This study was performed to evaluate the diagnostic usefulness of double-phase Tc-99m MIBI parathyroid scintigraphy with single photon emission computed tomography (SPECT) in patients with hyperparathyroidism. We also evaluated the relationship between Tc-99m MIBI uptake and oxyphil cell contents in parathyroid glands. The subjects were 28 parathyroid glands of 10 patients who underwent Tc-99m MIBI parathyroid scintigraphy and parathyroidectomy for clinically suspected hyperparathyroidism. Early and delayed pinhole images were obtained at 15 minutes and 2 hours after injection of Tc-99m MIBI, and SPECT images were followed. The weight and oxyphil cell contents of parathyroid tissue were obtained from pathologic specimen, and the scintigraphic findings were compared with histopathology. In surgical histopathology, 6 parathyroid adenomas and 9 parathyroid hyperplasias were confirmed. The sensitivity, specificity, and positive predictive value of early and delayed images were 46.7% (7/15), 76.9% (10/13), 70% (7/10) and 66.7% (10/15), 92.3% (12/13), 90.9% (10/11), respectively. SPECT image detected an additional small hyperplasia. The sensitivity, specificity, and positive predictive value of combined interpretation of early and delayed images with SPECT were 73.3% (11/15), 100% (13/13), 100% (11/11). The sensitivity was 100% (6/6) for adenoma, whereas that was 55.5% (5/9) for hyperplasia. Both adenomas and hyperplasias showed significantly increased oxyphil cell contents compared with normal parathyroid glands (p<0.0001), but the oxyphil cell content and weight were not significantly different between adenomas and hyperplasias. Double-phase Tc-99m MIBI parathyroid scintigraphy with SPECT is useful for lesion localization in patients with hyperparathyroidism. Although both adenoma and hyperplasia have increased oxyphil cell content, the sensitivity is high in adenoma, but low in hyperplasia.

  12. Skeletal changes after restoration of the euparathyroid state in patients with hypoparathyroidism and primary hyperparathyroidism.

    Science.gov (United States)

    Cipriani, Cristiana; Abraham, Alice; Silva, Barbara C; Cusano, Natalie E; Rubin, Mishaela R; McMahon, Donald J; Zhang, Chengchen; Hans, Didier; Silverberg, Shonni J; Bilezikian, John P

    2017-02-01

    Restoration of the euparathyroid state is associated with improvement of bone dynamics both in hypoparathyroidism and primary hyperparathyroidism. To date, no study has directly compared these two groups following correction of parathyroid hormone excess or deficiency. The study was designed to investigate changes in bone mineral density and trabecular bone score with restoration of the euparathyroid state by parathyroidectomy in primary hyperparathyroidism or recombinant parathyroid hormone [rhPTH(1-84)] replacement in hypoparathyroidism. This was a 2-year prospective intervention study in which we evaluated areal bone mineral density by DXA and trabecular bone score in 52 hypoparathyroid patients on rhPTH(1-84) replacement and 27 patients with primary hyperparathyroidism who underwent parathyroidectomy. We evaluated changes in areal bone mineral density by DXA and trabecular bone score at baseline, 6, 12, 18, and 24 months. After parathyroidectomy, areal bone mineral density increased from baseline at the lumbar spine and total hip at 6 months and at the femoral neck at 12 months, while there were no changes at the distal 1/3 radius. Treatment with rhPTH(1-84) was associated with significant increases in lumbar spine and decreases in distal 1/3 radius areal bone mineral density by 18 months in hypoparathyroid patients. At this time point, hypoparathyroid subjects demonstrated a significant increase in trabecular bone score from baseline, while there were no significant changes in trabecular bone score following parathyroidectomy. Bone mineral density increases both with administration of parathyroid hormone in a state of parathyroid hormone deficiency or removal of parathyroid hormone in a state of parathyroid hormone excess. However, only hypoparathyroid patients on rhPTH(1-84) appeared to have improvements in micro-architectural pattern as assessed by trabecular bone score.

  13. Preoperative parathyroid gland localization with technetium-99m sestamibi in secondary hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Pons, F.; Vidal-Sicart, S.; Fuster, D.; Herranz, R. [Department of Nuclear Medicine, Hospital Clinic, University of Barcelona (Spain); Torregrosa, J.V. [Unit of Renal Transplant, Hospital Clinic, Barcelona (Spain); Sabater, L.; Fernandez-Cruz, L. [Department of Surgery, Hospital Clinic, Barcelona (Spain)

    1997-12-01

    Technetium-99m sestamibi scintigraphy has become a valuable tool in locating parathyroid glands in patients with primary hyperparathyroidism. The aim of this study was to evaluate its usefulness in secondary hyperparathyroidism. Twenty patients were injected intravenously with 740 MBq of {sup 99m}Tc-sestamibi and images were obtained at 15 min and 2 h post injection. All patients underwent parathyroid ultrasonography (US) as well as bilateral surgical neck exploration and 64 parathyroid glands were removed. US revealed at least one enlarged gland in 15/20 patients (75%), while {sup 99m}Tc-sestamibi scintigraphy showed focal areas of increased uptake in at least one gland in 17/20 patients (85%). When imaging results for all glands were evaluated according to surgical results, sensitivity was 54% for parathyroid scintigraphy and 41% for US, and specificity was 89% for both imaging techniques. There was a discrepancy between the two imaging modalities in 28 glands (35%). The mean surgical weight of US-positive glands (1492{+-}1436 mg) was significantly higher than that of US-negative glands (775{+-}703 mg) (P<0.05). However, there were no significant differences in weight between sestamibi-positive and sestamibi-negative glands. When only sestamibi-positive glands were considered, a positive correlation between uptake and weight was found (r=0.4, P<0.05). In conclusion, parathyroid US and {sup 99m}Tc-sestamibi scintigraphy are complementary imaging techniques in the preoperative localization of abnormal parathyroid glands in patients with secondary hyperparathyroidism. The limited sensitivity of the techniques means that patients will still require bilateral neck exploration; therefore routine preoperative parathyroid scanning in renal patients is not justified. (orig.) With 3 figs., 2 tabs., 22 refs.

  14. Incidence of and risk factors for hungry bone syndrome in 84 patients with secondary hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Latus J

    2013-07-01

    Full Text Available Joerg Latus,1 Meike Roesel,1 Peter Fritz,2 Niko Braun,1 Christoph Ulmer,3 Wolfgang Steurer,3 Dagmar Biegger,4 M Dominik Alscher,1 Martin Kimmel1 1Department of Internal Medicine, Division of Nephrology, Robert Bosch Hospital, Stuttgart, Germany; 2Department of Diagnostic Medicine, Robert Bosch Hospital, Stuttgart, Germany; 3Department of Surgery, Robert Bosch Hospital, Stuttgart, Germany; 4Margarete Fischer-Bosch Institute of Clinical Pharmacology, University of Tuebingen, Stuttgart, Germany Introduction: Secondary hyperparathyroidism develops in nearly all patients with end-stage renal disease. Parathyroidectomy is often performed when medical therapy fails. The most common postoperative complication, hungry bone syndrome (HBS, requires early recognition and treatment. Materials and methods: A total of 84 patients who underwent parathyroidectomy because of secondary hyperparathyroidism were investigated. Detailed analysis of laboratory parameters (calcium, phosphate, parathyroid hormone, hemoglobin, and urea levels and baseline characteristics (age at time of surgery, duration of renal replacement therapy, and medication was performed to detect preoperative predictors for the development of HBS. Results: Average overall follow-up of the cohort was 4.7 years. Within this time frame, 13 of 84 patients had to undergo a second surgery because of recurrent disease, and HBS occurred in 51.2%. Only decreased preoperative calcium levels and younger age at time of surgery were significant predictors of HBS. Minimal levels of calcium were detected 3 weeks after surgery. Preoperative vitamin D therapy could not prevent HBS and could not shorten the duration of intravenous calcium supplementation. Conclusion: HBS is a very common complication after parathyroidectomy. Younger patients and patients with low preoperative calcium levels were at higher risk for the development of HBS. Remarkably, preoperative vitamin D therapy could not prevent HBS and had no

  15. A Randomized Study Comparing Parathyroidectomy with Cinacalcet for Treating Hypercalcemia in Kidney Allograft Recipients with Hyperparathyroidism.

    Science.gov (United States)

    Cruzado, Josep M; Moreno, Pablo; Torregrosa, José V; Taco, Omar; Mast, Richard; Gómez-Vaquero, Carmen; Polo, Carolina; Revuelta, Ignacio; Francos, José; Torras, Joan; García-Barrasa, Arantxa; Bestard, Oriol; Grinyó, Josep M

    2016-08-01

    Tertiary hyperparathyroidism is a common cause of hypercalcemia after kidney transplant. We designed this 12-month, prospective, multicenter, open-label, randomized study to evaluate whether subtotal parathyroidectomy is more effective than cinacalcet for controlling hypercalcemia caused by persistent hyperparathyroidism after kidney transplant. Kidney allograft recipients with hypercalcemia and elevated intact parathyroid hormone (iPTH) concentration were eligible if they had received a transplant ≥6 months before the study and had an eGFR>30 ml/min per 1.73 m(2) The primary end point was the proportion of patients with normocalcemia at 12 months. Secondary end points were serum iPTH concentration, serum phosphate concentration, bone mineral density, vascular calcification, renal function, patient and graft survival, and economic cost. In total, 30 patients were randomized to receive cinacalcet (n=15) or subtotal parathyroidectomy (n=15). At 12 months, ten of 15 patients in the cinacalcet group and 15 of 15 patients in the parathyroidectomy group (P=0.04) achieved normocalcemia. Normalization of serum phosphate concentration occurred in almost all patients. Subtotal parathyroidectomy induced greater reduction of iPTH and associated with a significant increase in femoral neck bone mineral density; vascular calcification remained unchanged in both groups. The most frequent adverse events were digestive intolerance in the cinacalcet group and hypocalcemia in the parathyroidectomy group. Surgery would be more cost effective than cinacalcet if cinacalcet duration reached 14 months. All patients were alive with a functioning graft at the end of follow-up. In conclusion, subtotal parathyroidectomy was superior to cinacalcet in controlling hypercalcemia in these patients with kidney transplants and persistent hyperparathyroidism.

  16. Diabetes due to recurrent pancreatitis secondary to hypercalcemia due to primary hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Sumit Kumar Chakrabarti

    2013-01-01

    Full Text Available Acute pancreatitis due to hypercalcemia associated with hyperparathyroidism (HPT is not very common. We herein report a case of a 21-year-old woman, who presented with acute pancreatitis. She had a past history of recurrent nephrolithiasis. Subsequent evaluation revealed hypercalcemia (serum calcium: 12.6 mg/dL; low phosphate (2.9 mg/dL with elevated parathyroid hormone (PTH, 156.7 pg/mL and HbA1c (6.9%. Diagnosis of primary HPT (PHPT was made. Recurrent pancreatitis due to hypercalcemia may have resulted in diabetes mellitus.

  17. NaF18-PET/CT imaging of second hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Win, Aung Zaw; Aparici, Carina Mari [San Francisco VA Medical Center, San Franciso (United States)

    2015-12-15

    The patient was a 59-year-old man with a history of hypertension and end-stage renal disease for 14 years. An NaF-18 positron emission tomography/CT bone scan was ordered to rule out osteosarcoma or other possible bone malignancies. A lesion representing a brown tumor was observed on the left femoral shaft. The incidence of ESRD is about 400 cases per million in the United States and it has risen fastest in older individuals. This is the second paper to report the use of NaF18-PET/CT to image secondary hyperparathyroidism, osteomalacia, mixed renal ostedystrophy and adyanmic bone disease.

  18. Metabolic acidosis-induced hypercalcemia in an azotemic patient with primary hyperparathyroidism.

    Science.gov (United States)

    Rastegar, Mandana; Levine, Barton S; Felsenfeld, Arnold J

    2014-06-01

    A 58-year-old man with Stage 3b chronic kidney disease and primary hyperparathyroidism treated with cinacalcet was admitted for acute cholecystitis. A cholecystostomy tube was placed, estimated glomerular filtration rate decreased, metabolic acidosis developed and ionized calcium increased from 1.33 to 1.76 mM despite cinacalcet administration. A sodium bicarbonate infusion corrected the metabolic acidosis restoring ionized calcium to normal despite no improvement in renal function. The correlation between the increase in serum bicarbonate and decrease in ionized calcium was r = -0.93, P metabolic acidosis increasing calcium efflux from bone while renal failure decreased the capacity to excrete calcium.

  19. Effect of paricalcitol on mineral bone metabolism in kidney transplant recipients with secondary hyperparathyroidism.

    Science.gov (United States)

    Borrego Utiel, Francisco José; Bravo Soto, Juan Antonio; Merino Pérez, María José; González Carmelo, Isabel; López Jiménez, Verónica; García Álvarez, Teresa; Acosta Martínez, Yelenei; Mazuecos Blanca, María Auxiliadora

    2015-01-01

    Secondary hyperparathyroidism is highly prevalent in kidney transplant recipients, and commonly results in hypercalcaemia; an association to osteopenia and bone fractures has also been observed. Paricalcitol has proved effective to control secondary hyperparathyroidism in chronic kidney disease in both dialysed and non-dialysed patients, with a low hypercalcaemia incidence. Currently available experience on paricalcitol use in kidney transplant recipients is scarce. Our main aim was to show the effect of paricalcitol on mineral bone metabolism in kidney transplant recipients with secondary hyperparathyroidism. A retrospective multicentre study in kidney transplant recipients aged>18 years with a 12-month or longer post-transplantation course, stable renal function, having received paricalcitol for more than 12 months, with available clinical follow-up for a 24-month period. A total of 69 patients with a 120 ± 92-month post-transplantation course were included. Baseline creatinine was 2.2 ± 0.9 mg/dl y GFR-MDRD was 36 ± 20 ml/min/1.73 m(2). Paricalcitol doses were gradually increased during the study: baseline 3.8 ± 1.9 μg/week, 12 months 5.2 ± 2.4 μg/week; 24 months 6.0 ± 2.9 μg/week (P10mg/dl showed gradually decreasing levels. Fifteen (21.7%) patients had received prior calcitriol therapy. When shifted to paricalcitol, such patients required paricalcitol doses significantly larger than those not having received calcitriol. Paricalcitol was used concomitantly to cinacalcet in 11 patients with significant PTH reductions being achieved; clinical course was similar to other patients and paricalcitol doses were also similar. Paricalcitol is an effective therapy for secondary hyperparathyroidism in kidney transplant recipients. Overall, no significant changes were observed in calcium and phosphorus levels or urinary excretion. Patients having previously received calcitriol required higher paricalcitol doses. When used in patients receiving cinacalcet

  20. Primary hyperparathyroidism in a child: The musculoskeletal manifestations of a late presenting rare endocrinopathy

    Directory of Open Access Journals (Sweden)

    Tamer Ahmed EL-Sobky

    2016-12-01

    Full Text Available Primary hyperparathyroidism (PHPT is rare in children and adolescents, but has greater morbidity in this age group. Most of these patients show predominantly skeletal pathology and to a lesser extent renal involvement. Osteopenia, osteoporosis and subperiosteal resorption are frequently encountered radiographic skeletal signs. This study describes the orthopedic manifestations of PHPT in a child. PHPT in this child exhibited a late presentation with significant clinical morbidity and extensive radiographic manifestations. The characteristic radiographic pattern of PHPT in childhood is an important contributor to the diagnosis. The radioclinical and biochemical correlations augment diagnostic accuracy and delineate extent of skeletal pathology.