Application of local sodium citrate anticoagulation in the initial speed of plasma exchange therapy / 重庆医学

Objective To study the initial pumping speed of sodium citrate in single plasma exchange with regional citrate anticoagulation(RAC).Methods From January to December 2021,15 patients and 67 times of treatment with local sodium citrate anticoagulation single plasma exchange in the hospital were in-cluded in the study.According to the initial pumping speed of sodium citrate,they were included in the low-speed group(n=33)and the high-speed group(n=34).The transmembrane pressure,filter pressure drop and venous pressure were compared between the two groups at 30 minutes,one hour and two hours after treatment.The free calcium concentration after plasma separator at 15 minutes and one hour after treatment,and the coagulation of plasma separator and extracorporeal circulation pipeline at the end of treatment were compared between the two groups.The concentration of free calcium,blood gas analysis and electrolyte were compared at the beginning of treatment,one hour after treatment and at the end of treatment.Results The free calcium concentration after the filter was monitored at 15 minutes and one hour of treatment in both groups was within the effective range of anticoagulation recommended by the guidelines.There were no lips,fingertip numbness and hand-foot convulsions in the two groups during the treatment,and no bleeding oc-curred after the treatment.There were four cases of hypocalcemia and two cases of alkalosis in the low-speed group,and 13 cases of hypocalcemia and eight cases of alkalosis in the high-speed group.The difference be-tween the two groups was statistically significant(P＜0.05).There were 15 cases of grade Ⅰ coagulation and five cases of grade Ⅱ coagulation in plasma separator and pipeline in the low-speed group,while there were 14 cases of grade Ⅰ coagulation and four cases of grade Ⅱ coagulation in plasma separator and pipeline in the high-speed group.There was no significant difference between the two groups(P＞0.05).Conclusion In plasma exchange treatment,according to the low initial pumping speed,RAC can not only ensure the anticoagulant effect,but also reduce the incidence of complications such as hypocalcemia and alkalosis.

Assessing Symptomatic Hypocalcemia Risk After Total Thyroidectomy: A Prospective Study

Abstract Introduction The most common postoperative complication of total thyroidectomy is hypocalcemia, usually monitored using serum parathyroid hormone and calcium values. Objective To identify the most accurate predictors of hypocalcemia, construct a risk assesment algorithm and analyze the impact of using several calcium correction formulas in practice. Methods A prospective, single-center, non-randomized longitudinal cohort study on 205 patients undergoing total thyroidectomy. Parathyroid hormone, serum, and ionized calcium were sampled post-surgery, with the presence of symptomatic or laboratory-verified asymptomatic hypocalcemia designated as primary outcome measures. Results Parathyroid hormone sampled on the first postoperative day was the most sensitive predictor of symptomatic hypocalcemia development (sensitivity 80.22%, cut-off value ≤ 2.03 pmol/L). A combination of serum calcium and parathyroid concentration sampled on the first postoperative day predicted the development of hypocalcemia during recovery with the highest sensitivity and specificity (94% sensitivity, cut-off ≤2.1 mmol/L, and 89% specificity, cut-off ≤1.55 pmol/L, respectively). The use of algorithms and correction formulas did not improve the accuracy of predicting symptomatic or asymptomatic hypocalcemia. Conclusions The most sensitive predictor of symptomatic hypocalcemia present on the fifth postoperative day was PTH sampled on the first postoperative day. The need for algorithms and correction formulas is limited.

Thyroid surgery in children: a single-center experience of 20 years

SUMMARY OBJECTIVE: Thyroidectomy is a relatively uncommon procedure in pediatric patients. We aimed to review our 20-year experience of thyroid surgery. METHODS: A total of 39 patients who underwent thyroid surgery from 2003 to 2023 were retrospectively evaluated. All patients were followed preoperatively and postoperatively by our institutional multidisciplinary board. Patients were divided into two groups based on their pathologies: benign and malignant. RESULTS: In total, 39 patients (27 girls and 12 boys) underwent 47 thyroid surgeries (total thyroidectomy in 19 patients and subtotal thyroidectomy in 20 patients, with 8 of them having completion thyroidectomy). Notably, 20 (51%) patients had benign and 19 (49%) patients had malignant pathologies. Median age at operation was 157 (9-223) months in the benign group and 182 (1-213) months in the malignant group. In the benign group, 12 (60%) patients had colloidal goiter and 8 (40%) patients had other conditions. In the malignant group, 12 (63%) patients had papillary thyroid carcinoma, 3 (16%) patients had follicular thyroid carcinoma, 2 (11%) had medullary thyroid carcinoma, and 2 patients had other thyroid malignancies. Overall permanent complication rate was 2 out of 39 (5%), which was similar for both groups (1 hypocalcemia in each group). The median follow-up was 38 months (1-179 months) with no local recurrence or distant metastasis. CONCLUSION: Pediatric thyroidectomies are performed on a heterogeneous group of pediatric patients due to a diverse group of pathologies. A multidisciplinary approach is required for proper initial management and surgical strategy with decreased complication rate and event-free survival of these patients in experienced tertiary centers.

Síndrome de hueso hambriento en pacientes con paratiroidectomía

Introducción: El síndrome de hueso hambriento (SHH) es una complicación asociada a la realización de una paratiroidectomía, definido por la persistencia de más de 4 días de hipocalcemia (Calcio sérico menor a 8,5 mg/dl) con requerimiento de suplementación de calcio endovenoso. Este es el primer estudio realizado en Colombia, que tiene el objetivo de precisar la prevalencia de SHH en un Hospital de referencia en Latinoamérica. Metodología: Se realizó un estudio de corte transversal retrospectivo, se revisaron los registros de historias clínicas de pacientes con antecedente de hiperparatiroidismo primario, secundario y terciario que requirieron paratiroidectomía parcial o total en el Hospital de San José de Bogotá, entre Enero del 2013 y Diciembre de 2021. Resultados: Se identificaron 247 pacientes con diagnóstico de hiperparatiroidismo, la mayoría de los pacientes (65,1%) fueron hombres, con una edad mediana de 50,9 años (rango de 25 a 71 años). La prevalencia del SHH fue del 11,7%, con una relación mujer-hombre de 1.5:1, no hubo casos de patología maligna. Los niveles de hormona paratiroidea séricos preoperatorios tuvieron una correlación estadísticamente significativa con los niveles de hormona paratiroidea postquirúrgica. Conclusiones: El síndrome de hueso hambriento es una complicación que puede presentarse posterior a la realización de una paratiroidectomía. Este estudio presenta una prevalencia más baja que la descrita en la literatura.

Introduction: The Hungry Bone Syndrome (HBS) is a rare complication associated with parathyroidectomy, defined as hypocalcemia (serum calcium less than 8,5 mg/dl) that persist more than 4 days and requires intravenous calcium supplementation. This is the first study made in Colombia with the objective to determine the prevalence of HBS in a reference hospital in Latin America. Methods: A retrospective cross-sectional study was conducted; medical records were reviewed from patients with medical history of primary, secondary, and tertiary hyperparathyroidism that require partial or total parathyroidectomy in San Jose Hospital in Bogota, between January 2013 and December 2021. Results: 247 patients were identified with hyperparathyroidism, most patients were males (65.1%), with a mean age of 50.9 years old (range 25-71 years old). The prevalence of HBS was 11.7%, with a ratio women-men 1.5:1, no malignant pathology was described. Preoperative parathyroid hormone levels and postoperative parathyroid hormone levels were statistically significant. Conclusions: The Hungry Bone Syndrome is a complication that can be present after a parathyroidectomy. This study presents a lower prevalence than the literature described.

Severe Hypocalcemia as an Atypical Manifestation of Seronegative Celiac Disease in a Patient with Systemic Lupus Erythematosus: Case Report

Aim: To describe the clinical picture and diagnosis of an episode of severe hypocalcemia in a patient with systemic lupus erythematosus (SLE) in remission, with chronic diarrhea that led to the diagnosis of celiac disease (CD). Case presentation: 22-year-old patient, diagnosed with SLE at age 10, in remission. He consulted for a two-month history of paresthesias, muscle spasms, myalgias, and episodes of tetany in the previous week, associated with malabsorptive postprandial diarrhea. His laboratory tests showed severe hypocalcemia, vitamin D deficiency, severe hypomagnesemia, and mild hypokalemia. In the study for CD, serology reported normal anti-tissue transglutaminase IgA, antigliadin IgA, and IgG and low total IgA levels. Enteroscopy revealed erosive bulbo-duodenitis, with villous atrophy and increased intraepithelial lymphocytes in the pathology, which, together with the immunohistochemical study, allowed the diagnosis of CD, Marsh 3a type. Management was initiated with a gluten-free diet, with a positive clinical response. Conclusion: CD should be suspected in patients with SLE who present with diarrhea, abdominal pain, nausea/vomiting, recurrent oral aphthosis, and anemia. In rare seronegative CD cases, other causes of villous atrophy, mainly infectious, toxic, and immunological, must be ruled out. There is an association between CD and SLE, so diagnosis must be early and timely with the best testing scheme available to achieve effective treatment before complications occur.

Objetivo: Describir la presentación clínica y diagnóstico de un episodio de hipocalcemia grave en un paciente con lupus eritematoso sistémico (LES) en remisión, con diarrea crónica que condujo al diagnóstico de enfermedad celíaca (EC). Presentación del caso: Paciente de 22 años, con diagnóstico de LES a los 10 años, en remisión. Consultó por 2 meses de evolución de parestesias, espasmos musculares, mialgias y episodios de tetania en la semana previa, asociado a diarrea posprandial malabsortiva. Sus laboratorios mostraron hipocalcemia grave, déficit de vitamina D, hipomagnesemia grave e hipopotasemia leve. En el estudio para EC la serología reportó antitransglutaminasa tisular IgA, antigliadina IgA e IgG normales y niveles de IgA total bajos. La enteroscopia evidenció bulboduodenitis erosiva, con atrofia de las vellosidades y aumento de linfocitos intraepiteliales en la patología, que junto al estudio inmunohistoquímico permitieron concluir el diagnóstico de EC, tipo Marsh 3a. Se inició el manejo con una dieta libre de gluten, con una respuesta clínica positiva. Conclusión: La EC debe sospecharse en pacientes con LES que inicien con diarrea, dolor abdominal, náuseas/vómitos, aftosis oral recurrente y anemia. En los casos de EC seronegativa, los cuales son poco comunes, se deben descartar otras causas de atrofia de las vellosidades principalmente infecciosas, tóxicas e inmunitarias. Existe una asociación entre EC y LES, por lo cual el diagnóstico debe ser precoz y oportuno con el mejor esquema de pruebas disponible para lograr realizar un tratamiento efectivo antes de que se presenten las complicaciones.

Incidencia de Hipocalcemia Post Tiroidectomía. Factores predictores (PTH, Fósforo, Magnesio) y protocolo de manejo con alta precoz

Se ha propuesto la medición de PTH como predictor de hipocalcemia postoperatoria transitoria y permanente. No hay un estándar de punto de corte o tiempo de toma de muestra. El objetivo es reportar la incidencia de hipocalcemia post tiroidectomía en un grupo quirúrgico de alto volumen y proponer un protocolo de manejo ambulatorio (esquemas de I a V) según niveles postoperatorios (PO) de calcemia, PTHi (normal, baja o indetectable) y síntomas. Y determinar valores de PTH postoperatoria como predictores de hipocalcemia. En 106 pacientes con tiroidectomía total entre 2019 y 2021 se realiza medición de niveles pre y postoperatorios de calcio, magnesio, fosforo y PTHi. Se observó 29% y 1% de hipocalcemia transitoria ( 12 meses). Los puntos de corte para predecir hipocalcemia fueron PTH < 8,8 pg/ml y < 80% de descenso de % de PTH (d % PTH) al día siguiente. Con el manejo propuesto se indica el alta precoz (promedio 1.05 días) y el costo de la prescripción es acotado. No hay asociación significativa de hipomagnesemia e hiperfosfemia PO con la hipocalcemia PO. El tratamiento más utilizado es de carbonato de calcio exclusivo (esquemas I y II). Los pacientes se mantienen con síntomas leves a las 2 semanas PO en 5% y logran suspender el tratamiento vía oral en 93% en este mismo período. Los protocolos de medición de PTH como predictor de hipocalcemia son muy variados. Cada centro debe conocer y establecer sus propios protocolos de manejo. Con esta experiencia demostramos la utilidad y seguridad de un esquema de manejo basado en calcemia, PTH (normal, baja o indetectable) y síntomas, con indicación de tratamiento profiláctico para todos los pacientes y ajuste ambulatorio seguro y de menor costo que una hospitalización prolongada.

Introduction: PTH measurement has been proposed to predict transient and permanent postoperative hypocalcemia. There is no standard cut-off point or time for sampling. Aim: To report the incidence of post-thyroidectomy hypocalcemia in a high-volume surgical group and propose an outpatient management protocol according to postoperative (PO) calcium levels, iPTH (normal, low, or undetectable), and symptoms. Furthermore, determine postoperative PTH values as predictors of hypocalcemia. Methods: In 106 patients with total thyroidectomy between 2019 and 2021, pre-and postoperative levels of calcium, magnesium, phosphorus, and iPTH were measured. Results: Transient ( 12 months) hypocalcemia was observed in 29% and 1%. Cut-off points to predict hypocalcemia were PTH < 8.8 pg/mL and < 80% decrease in % PTH (d% PTH) the day after surgery. With the proposed management, early discharge is indicated (an average of 1.05 days), and the prescription cost is limited. There is no significant association between PO hypomagnesemia and hyperphosphatemia with PO hypocalcemia. The most widely used treatment is exclusive calcium carbonate (schemes I and II). Patients remain with mild symptoms at two weeks PO in 5% and discontinue oral treatment in 93% in this period. Conclusions: Protocols for measuring PTH as a hypocalcemia predictor vary. Each center must know and establish its management protocols. With this experience, we demonstrate the usefulness and safety of a management scheme based on calcium, PTH (normal, low, or undetectable), and symptoms with an indication of prophylactic treatment for all patients and a safe outpatient setting at a lower cost than prolonged hospitalization.

Postoperative serum magnesium levels as a predictor for the need for calcium replacement after total thyroidectomy: a prospective study

ABSTRACT Objective: Our aim was to assess the ability of serum magnesium (Mg), measured on the first postoperative day (Mg1PO), to predict the need for calcium (Ca) replacement in patients undergoing total thyroidectomy (TT). Subjects and methods: Eighty patients undergoing TT, with Mg1PO and PTH dosage in the first (PTH1h) and eighth (PTH8h) hours after TT, were evaluated for the need for Ca replacement. Data were evaluated by uni/multivariate logistic regression and Receiver Operating Characteristic (ROC) curve. Results: 32 patients (40%) required Ca replacement. Median PTH1h, PTH8h and Mg1PO were higher in the no replacement group: 17 versus (vs) 3 pg/mL (p < 0.001), 18.2 vs 3.0 pg/mL (p < 0.001) and 2 vs 1.6 mg/dL (p < 0.001), respectively. Mg1PO was the isolated predictor for this replacement (odds ratio = 0.0004, 95% confidence interval: 0.000003-0.04; p = 0.001), with the cut-off value of 1.8 mg/dL showing sensitivity and specificity of 78.1% and 87.5%, respectively. Conclusions: In this group of patients, serum Mg1PO was the isolated predictor for the need for Ca replacement.

A RETROSPECTIVE STUDY OF CORRELATION BETWEEN SERUM CREATININE, SERUM CALCIUM, SERUM INORGANIC PHOSPHORUS AND SERUM MAGNESIUM LEVELS IN PATIENTS ATTENDING NEPHROLOGY CLINICS IN TERTIARY CARE CENTER

Introduction: Chronic kidney disease (CKD) has been recognized as a leading public health problem worldwide. The global estimated prevalence of CKD is 13.4%. Kidney plays a critical role in regulating serum levels of Calcium, Magnesium and Phosphate. Calcium, phosphorus and magnesium homeostasis is altered in chronic kidney disease(CKD). Objective Objective: of the study is to establish the correlation of increase in serum creatinine levels with Calcium, Magnesium and Phosphate levels in patients. Design & Methods: This observational retrospective study included patients attending nephrology department in tertiary care centre for a period of two months. In total, 229 patients' data was accessed from the medical records. Patients were grouped in 5 groups based on serum Results: creatinine levels. Group I with serum creatinine levels of 0.5mg/dl to 0.8mg/dl, group II with serum creatinine levels of 0.8 to 1.1mg/dl, group III with serum creatinine levels of 1.1 to 3mg/dl, group IV with serum creatinine levels of 3 to 6mg/dl, group V with serum creatinine levels above 6mg/dl. Overall from group I to Group V , Serum Calcium showed negative and significant correlation, Serum phosphorus levels showed positive and significant correlation, Serum magnesium showed negative and insignificant correlation with serum creatinine. As Conclusions: serum creatinine increases, more patients show decline in serum calcium levels and serum magnesium levels and increase in serum inorganic phosphorus levels. Hypocalcemia, hypomagnesemia and hyperphosphatemia have been associated with increased risk of cardiovascular morbidity and mortality in cases of CKD. Hypomagnesemia has possible association with rapid decline in kidney function. Therefore, regular analysis of these parameters is important for predicting the prognosis of CKD and cardiovascular risk in cases of CKD.

Postoperative longitudinal changes of serum calcium level and its influencing factors in patients with primary hyperparathyroidism / 中华普通外科杂志

Objective:To compare the changes of serum calcium level before and after surgical resection in patients with primary hyperparathyroidism.Methods:Two hundred and seventy-one patients with primary hyperparathyroidism were enrolled from Dec 1992 to Dec 2020 in Beijing Jishuitan Hospital. Serum calcium concentrations were measured before operation, 20 min during surgery, then 2 weeks 1-6 months , 7-12 months and 1 year respectively after operation. The baseline data of postoperative serum calcium such as sex, age, other genetic endocrine diseases, osteopathia and urolithiasis were calculated. The generalized estimation equation was used to analyze the changes of serum calcium in different types of patients before and after operation.Results:The most common postoperative hypocalcemia occurred within 2 weeks, and it occurred frequently half a year after surgery. There was no significant difference in blood calcium between male patients ( t=0.875, P=1.000) and patients with bone lesions ( t=0.034, P=3.049) from 1 to 6 months after surgery and 2 weeks after surgery. Blood calcium level in patients aged 15-35 years old from 1 to 6 months ( t=0.239, P=1.000) , from 7 to 12 months ( t=1.380, P=0.935) and 2 weeks after surgery was not statistically different. The change of bone mineral density was correlated with the change of blood calcium after operation ( F=6.895, P=0.004). Conclusions:The incidence of hypocalcemia was the highest in patients with hyperparathyroidism 2 weeks after surgery, and the blood calcium level was stable within the normal range 1 year later. The blood calcium value of male patients was still at a lower level than that of female patients within six months after surgery. In patients with bone disease, the blood calcium value was lower and recovered slowly 2 weeks after surgery. The blood calcium value of patients aged 15-35 was at a low level within 1 year after surgery.

Paroxysmal kinesigenic dyskinesia secondary to pseudohypoparathyroidism: a report of 2 cases and literature review / 中华神经科杂志

Objective:To explore the clinical characteristics of paroxysmal kinesigenic dyskinesia (PKD) secondary to pseudohypoparathyroidism (PHP), so as to improve the clinicians′ understanding of the disease.Methods:The clinical data of 2 cases of PKD secondary to PHP in Beijing Tiantan Hospital, Capital Medical University from June 2022 to October 2022 were summarized, and the related literature was reviewed to analyze the pathogenesis, clinical manifestations, laboratory examination and imaging characteristics of the disease, as well as its treatment and prognosis.Results:Ten cases of PKD secondary to PHP were previously reported. A total of 12 cases (including these 2 cases) were included in the study, of which 7 were males (7/12) and 5 were females (5/12). The onset age was 8-23 years. The clinical manifestations of the disease included paroxysmal limb torsion, limb stiffness, limb tremor, throwing movements, and dance like movements. There were 3 cases (3/12) who had seizure at the same time. Ten cases (10/12) were induced by exercise, while 2 cases (2/12) were not mentioned the inducing factor. Eleven cases (11/12) with paroxysmal symptoms lasting less than 2 minutes can be relieved automatically. Only 1 case had a family history. Laboratory examination of all patients showed low calcium, high phosphorus and significant elevation of parathyroid hormone. The head CT scans of 10 cases (10/12) showed multiple intracranial calcifications. There were 9 cases (9/12) alone received the supplementation of calcium and active vitamin D, while 3 cases (3/12) were additionally treated with antiepileptic drugs. All cases had good prognosis.Conclusions:PHP is a rare cause of secondary PKD. A small number of patients can have seizures at the same time. Most patients have a good prognosis after receiving calcium and active vitamin D supplementation.

Valor pronóstico de la parathormona postoperatoria de 24 horas en el manejo de la hipocalcemia tras tiroidectomía total en un hospital de Valencia durante 2012-2019 / Prognostic value of 24-hour postoperative parathormone in the management of hypocalcemia after total thyroidectomy in a hospital in Valencia during 2012-2019

Introducción: La hipocalcemia por hipoparatiroidismo es la complicación más frecuente tras tiroidectomía total. Un factor predictor importante de hipocalcemia es la parathormona postoperatoria, pero el momento en el que otorga mejores resultados predictivos aún se discute. Objetivo: El objetivo es analizar el valor pronóstico de la parathormona postoperatoria a las 24 horas como indicador de hipocalcemia, en comparación con la seriación de los niveles de calcio. Metodología: Estudio observacional retrospectivo y descriptivo de 297 pacientes intervenidos de tiroidectomía total durante ocho años. Los pacientes fueron clasificados en tres grupos de riesgo según la parathormona postoperatoria (alto, medio y bajo riesgo). Para comparar la parathormona frente al calcio postoperatorio como predictor de hipocalcemia, se obtuvieron curvas ROC y áreas debajo de la curva. Resultados: El riesgo relativo de tener hipocalcemia con parathormona ≤15 pg/mL es de 353,4 (p = 0,00). La prueba de parathormona postoperatoria (≤15 pg/mL a las 24 h) obtuvo una sensibilidad del 96,25 % para la detección de hipocalcemia, especificidad del 94,06 % y precisión global del 95,03 %. El grupo de alto riesgo (parathormona ≤15 pg/mL) concentra la mayoría de los pacientes con hipocalcemia, y abarca la totalidad de los casos permanentes. Conclusiones: La parathormona postoperatoria a las 24 horas de la tiroidectomía total es un test con un valor pronóstico considerable, capaz de predecir el riesgo de hipocalcemia postquirúrgica. Se encontró que los pacientes con parathormona >15 pg/mL pueden ser dados de alta de manera segura.

Introduction: Hypocalcemia due to hypoparathyroidism is the most frequent complication after total thyroidectomy. An important predictive factor of hypocalcaemia is postoperative parathormone (PTH), but the optimal time for testing PTH levels is under discussion. Objectives: The objective is to analyze the prognostic value of postoperative PTH at 24 hours as an indicator of hypocalcaemia, compared to serum calcium levels. Methodology: Descriptive retrospective observational study of 297 patients who underwent total thyroidectomy over an 8-year period. The patients were classified into 3 risk groups according to postoperative parathormone (high, medium and low risk). To compare parathormone versus postoperative calcium as a predictor of hypocalcemia, ROC curves and areas under the curve (AUC) were obtained. Results: The relative risk of having hypocalcemia with parathormone ≤ 15 pg/mL is 353.4 (p = 0.00). The postoperative parathormone test (≤ 15 pg / mL at 24h) obtained a sensitivity of 96.25% for the detection of hypocalcemia, specificity of 94.06% and global precision of 95.03%. The high-risk group (parathormone ≤ 15 pg/mL) accounts for the vast majority of patients with hypocalcemia and covers all permanent cases. Conclusions: Postoperative levels 24 hours after total thyroidectomy is a test with considerable prognostic value, capable of predicting the risk of postsurgical hypocalcemia. Patients with levels over > 15 pg/mL can be safely discharged.

Pseudohypoparathyroidism: an endocrine orphan disease. case report

Introduction: Pseudohypoparathyroidism (PHP) is a heterogeneous group of rare endocrine disorders characterized by resistance to the parathyroid hormone. There are few reports on PHP in Colombia, so the publication of the present case contributes to increase the interest in its research in the clinical setting. Case presentation: An 18-year-old male with a history of hypothyroidism diagnosed at 4 months of age, stunted growth, delayed puberty, obesity, brachydactyly, pathologic fractures, femoral osteochondroma, insomnia, paresthesia, and chronic constipation was referred to the endocrinology service of a tertiary care center in Bogotá (Colombia) after being hospitalized following a seizure episode. On admission, laboratory tests revealed hypocalcemia, hyperphosphatemia, 25-hydroxy vitamin D in the range of insufficiency and elevated PTH. Imaging studies showed heterotopic ossifications and calcifications of the basal ganglia. A genetic study confirmed the diagnosis of PHP1A, so treatment was started with calcium, cholecalciferol and phosphorus chelators, leading to a satisfactory course. Both the patient and his first-degree relatives received genetic counseling and interdisciplinary assessment. Conclusion: Although PHP type 1A is an unrecognized complex genetic disorder, it has a critical clinical importance within the differential diagnoses of hypocalcemia. Without prompt diagnosis and treatment, patients may experience serious and potentially fatal metabolic consequences.

Introducción. El seudohipoparatiroidismo (SPT) es un trastorno genético poco frecuente que se caracteriza por la resistencia a la hormona paratiroidea (PTH). En Colombia existen pocos reportes sobre esta enfermedad, por lo que la publicación del presente caso contribuye a aumentar el interés en su búsqueda en el ámbito clínico. Presentación del caso. Hombre de 18 años con antecedente de hipotiroidismo (diagnosticado a los 4 meses de nacido), retraso del crecimiento, desarrollo puberal tardío, obesidad, braquidactilia, fracturas patológicas, osteocondroma femoral, insomnio, parestesias y estreñimiento crónico, quien asistió al servicio de endocrinología de un hospital de tercer nivel de Bogotá (Colombia) remitido luego de haber estado hospitalizado por un episodio convulsivo. En dicha hospitalización los laboratorios evidenciaron hipocalcemia, hiperfosfatemia y 25-OH vitamina D en rango de insuficiencia con niveles elevados de PTH, y los estudios imagenológicos demostraron osificaciones heterotópicas y calcificaciones de ganglios basales del cerebro. Al paciente se le realizó un estudio genético que confirmó el diagnóstico de SPT1A, por lo que se le inició manejo con suplencia de calcio, colecalciferol y quelantes de fósforo, con lo cual evolucionó satisfactoriamente. Tanto el paciente como sus familiares de primer grado recibieron asesoramiento y valoración interdisciplinaria. Conclusiones. El SPT1A es un trastorno genético complejo poco conocido pero de alta importancia clínica dentro de los diagnósticos diferenciales de hipocalcemia que debe considerarse ya que sin el diagnóstico y tratamiento oportunos, los pacientes pueden presentar consecuencias metabólicas graves y potencialmente fatales.

Hypoparathyroidism: update of guidelines from the 2022 International Task Force

ABSTRACT The 2022 International Task Force guidelines for chronic hypoparathyroidism will be published within several months in the Journal of Bone and Mineral Research. These guidelines update the original guidelines published in 2016, and include new information from literature published since then. Chronic postsurgical hypoparathyroidism is now defined as lasting for at least 12 months after surgery, rather than 6 months. Chronic postsurgical hypoparathyroidism may be predicted by serum PTH <10 pg/mL in the first 12-24 hours after surgery. The most common symptoms and complications of chronic hypoparathyroidism based on the literature are summarized in detail. How to monitor and manage patients with hypoparathyroidism is described in detail where recommendations can be given. These guidelines are intended to frame the diagnosis and care of patients with chronic hypoparathyroidism for at least the next five years.

Bicarbonato de sodio intravenoso ¿Cuándo, cómo y por qué utilizarlo? / Intravenous sodium bicarbonate. When, how and why to use it?

Severe metabolic acidosis is defined by a pH < 7.2 with HCO3− < 8 mE- q/L in plasma. Its best treatment is to correct the underlying cause. However, acidemia produces multiple complications such as resistance to the action of catecholamines, pulmonary vasoconstriction, impaired cardiovascular function, hyperkalemia, immunological dysregulation, respiratory muscle fatigue, neurological impairment, cellular dysfunction, and finally, it contributes to multisystemic failure. Intravenous NaHCO3 buffers severe acidemia, preventing the associated damage and gains time while the causal disease is corrected. Its indication requires a risk-benefit assessment, considering its complications. These are hypernatremia, hypokalemia, ionic hypocalcemia, rebound alkalosis, and intracellular acidosis. For this reason, therapy must be "adapted" and administered judiciously. The patient will require monitoring with serial evaluation of the internal environment, especially arterial blood gases, plasma electrolytes, and ionized calcium. Isotonic solutions should be preferred instead of hypertonic bicarbonate. The development of hypernatremia must be prevented, calcium must be provided for hypocalcemia to improve cardiovascular function. Furthermore, in mechanically ventilated patients, a respiratory response similar to the one that would develop physiologically, must be established to be able to extract excess CO2 and thus avoid intracellular acidosis. It is possible to estimate the bicarbonate deficit, speed, and volume of its infusion. However, the calculations are only for reference. More important is to start intravenous NaHCO3 when needed, administer it judiciously, manage its side effects, and continue it to a safe goal. In this review we address all the necessary elements to consider in the administration of intravenous NaHCO3, highlighting why it is the best buffer for the management of severe metabolic acidosis.

Enfermedad ósea metabólica del prematuro: revisión de tema / Metabolic bone disease of prematurity: topic review

Resumen La enfermedad ósea metabólica del prematuro es una patología multifactorial que representa una importante causa de morbilidad, cuya prevalencia ha aumentado. Su diagnóstico requiere criterios bioquímicos, radiológicos y, en etapas avanzadas, clínicos; por lo cual, muchos autores recomiendan estrategias de tamizaje y prevención. El objetivo del presente artículo es realizar una revisión de los aspectos más relevantes respecto a la enfermedad ósea metabólica del prematuro, con énfasis en la prevención y tratamiento precoz. Se realizó una revisión bibliográfica con términos MeSH, en las bases de datos de Pubmed, ClinicalKey, ScienceDirect, SciELO y LILACS. Aunque no hay consenso en las pautas de tamizaje, diagnóstico y tratamiento, la principal estrategia usada en la actualidad es el soporte nutricional individualizado que cubra las demandas de calcio, fósforo y vitamina D, asociado a métodos de intervención clínica y seguimiento de bebés de alto riesgo. La comprensión de esta patología permitirá mejorar las estrategias de tamización, diagnóstico precoz, y de esta forma evitará complicaciones.

Abstract Metabolic bone disease of prematurity is a multifactorial pathology that represents a significant cause of morbidity and has increased in prevalence. Its diagnosis requires biochemical, radiological, and, in advanced stages, clinical criteria; therefore, many authors recommend screening and prevention strategies. This article aims to review the most relevant aspects of the metabolic bone disease of prematurity, with emphasis on prevention and early treatment. A bibliographic review was carried out with MeSH terms in the Pubmed, ClinicalKey, ScienceDirect, SciELO, and LILACS databases. Although there is no consensus on screening, diagnosis and treatment guidelines, the main strategy currently used is to provide individualized nutritional support that covers the demands of calcium, phosphorus and vitamin D associated with clinical intervention methods and monitoring of high-risk babies. Understanding this pathology will improve screening strategies and early diagnosis and thus avoid complications.

Clinical management of primary hypomagnesemia with secondary hypocalcemia / 中华内分泌代谢杂志

Primary hypomagnesemia with secondary hypocalcemia(HSH) is a rare cause of hypoparathyroidism. This article presents a case of a 26-year-old male with recurrent generalized weakness and tetany, and a literature review of diagnosis and treatment of primary HSH. The biochemical tests revealed the patient had severe hypomagnesemia, mild hypocalcemia, hypokalemia, and hypoparathyroidism. Transient receptor potential melastatin-6(TRPM6) gene mutation were detected by gene test, which confirmed the diagnosis of primary HSH. The patient had been treated with long term oral magnesium supplementation, who remained asymptomatic during the follow-up. Primary HSH is a rare autosomal-recessive disorder caused by mutations in the TRPM6 gene which encoding a magnesium permeable channel expressed in the intestine and the kidney. The primary defect is impaired intestinal absorption of magnesium with secondary renal excretion, leading to a series of clinical symptoms. The treatment is mainly through lifelong magnesium supplementation.

Total thyroidectomy versus lobectomy for unilateral sporadic medullary thyroid carcinoma / 中华普通外科杂志

Objective:To compare the prognosis of patients with unilateral sporadic medullary thyroid carcinoma treated by different surgical selection, and analyze the independent risk factors affecting the prognosis.Methods:One hundred and twenty-six patients at Tianjin Medical University Cancer Institute and Hospital from Feb 2011 to Oct 2018 were retrospectively divided into group A (total thyroiclectomy) and group B (unilateral lobectomy).Results:There were no significant differences in postoperative recurrence rate ( χ2=0.394, P=0.530), mortality ( χ2=3.175, P=0.146), biochemical cure rate ( χ2=0.613, P=0.434), progression free survival and overall survival ( P=0.278, 0.175) between group A and group B; Tumor diameter ≥4 cm and lateral cervical lymph node metastasis were independent risk factors affecting the overall survival. The incidence of postoperative temporary hypocalcemia ( χ2=5.068, P=0.024) and permanent hypocalcemia ( χ2=6.590, P=0.010) in group A was higher than that in group B. Conclusions:Ipsolateral thyroidectomy can be applied to patients with unilateral sporadic medullary thyroid carcinoma with similar long term prognosis and tower incidence of temporary hypocalcemia and permanent hypocalcemia compared to total thyroidectomy.

Risk factors for hypocalcemia after parathyroidectomy in maintenance hemodialysis patients with secondary hyperparathyroidism / 中华肾脏病杂志

Objective:To explore the risk factors of hypocalcemia and the correlation between calcium supplementation and clinical parameters after parathyroidectomy (PTX) in maintenance hemodialysis patients with secondary hyperparathyroidism (SHPT), and to analyze the effect of calcium supplementation after PTX on the long-term prognosis of patients.Methods:This study was a single-center retrospective study. The patients who underwent PTX in maintenance hemodialysis patients with SHPT in the Huashan Hospital affiliated to Fudan University from October 2014 to March 2021 were retrospectively enrolled. Total PTX with auto transplantation or total PTX alone were the surgical procedures. According to the postoperative requirement of calcium in the first week, the patients were divided into two groups: high calcium supplement (>16.05 g/week) group and low calcium supplement group (≤16.05 g/week). According to the average serum calcium level in the first week after operation, the patients were divided into hypocalcemia group (≤2.1 mmol/L) and non-hypocalcemia group (>2.1 mmol/L) and the differences of clinical parameters between the two groups were compared. The correlation between clinical parameters and the postoperative calcium requirement was examined through Pearson or Spearman correlation analysis. The influencing factors for hypocalcemia after PTX were examined through logistic regression analysis. The survival curve was made by Kaplan-Meier method, and the difference of cumulative survival rate between the two groups was compared by log-rank test.Results:A total of 98 maintenance hemodialysis patients with SHPT were enrolled. The levels of serum calcium, phosphorus, and intact parathyroid hormone (iPTH) after the operation decreased significantly than those of preoperation (all P<0.05). Multiple linear regression analysis showed age ( β=-0.160, P=0.030), iPTH ( β=0.004, P=0.025) and C-reactive protein ( β=0.186, P=0.011) were correlated with postoperative calcium requirement. Preoperative alkaline phosphatase ( OR=1.002, 95% CI 1.000-1.004, P=0.018) and hemoglobin ( OR=0.977, 95% CI 0.954-1.000, P=0.048) independently predicted the occurrence risk of postoperative hypocalcemia through multivariate logistic regression analysis. The recurrence rate of high calcium supplement group was higher than that of low calcium supplement group (10.26% vs 0, P=0.023) and there was no significant difference in all-cause mortality between the two groups (17.95% vs 5.08%, P=0.086). The recurrence rate between the hypocalcemia group and non-hypocalcemia group was no significantly different (8.3% vs 1.8%, P=0.451) and there was no significant difference in all cause mortality between the two groups (12.5% vs 12.7%, P=1.000). Kaplan-Meier survival curve showed that the cumulative survival rate between the two groups was no significantly different (log-rank test χ2=0.147, P=0.702). Conclusions:PTX is a safe and effective therapeutic method to reduce the level of iPTH and improve the metabolism of calcium and phosphorus in SHPT patients. Age, iPTH and C-reactive protein are correlated with the postoperative requirement of calcium in the first week. Preoperative alkaline phosphatase and hemoglobin are independent risk factors for postoperative hypocalcemia. Correcting preoperative electrolyte disorder, improving infection and anemia can reduce the incidence of hypocalcemia after PTX.

A Case of Chronic Myelomonocytic Leukemia: the Onset of which was Manifestedby Hypokalemia and Hypocalcemia / 罕见病研究

Intractable hypokalemia and hypocalcemia are rare complications in leukemia patients. This article reports a patient with chronic myelomonocytic leukemia (CMML)with refractory hypokalemia, hypocalcemia, hypochloridemia, hypomagnesemia-a 48-year-old man complained of fatigue, nausea, and face numbness for over one month. The number of monocytosis in peripheral blood and bone marrow increased remarkably. At the same time, the patient developed hypokalemia, hypochloridemia, hypocalcemia, hypomagnesemia, metabolic alkalosis, massive proteinuria, and increase in plasma aldosterone and renin. After two coursea of treatment using Azacitidine therapy, complete remission of bone marrow was achieved and the electrolyte disturbance was almost corrected, except hypomagnesemia. We summarize the clinical characteristics of the patient so as to raise the clinical awareness of such cases.