Hypercalcium crisis and postoperative hungry bone syndrome caused by primary hyperparathyroidism: a case report / 临床耳鼻咽喉头颈外科杂志

To review the diagnosis and treatment of a case of hypercalcium crisis caused by primary hyperparathyroidism（PHPT） and prophylactic treatment of hungry bone syndrome. In a 32-year-old male with hypercalcemia, the main manifestations were loss of appetite, nausea, polyuria, polydipsia, fatigue, lethargy, etc. parathyroid hormone, serum calcium increased, thyroid function was normal, thyroid color ultrasound and MRI showed space-occupying behind the right thyroid, radionuclide examination showed abnormal imaging agent concentration in the right parathyroid area, there was a history of pathological fracture. Clinically diagnosed as hypercalcemia crisis secondary to PHPT.

QTc, Tp-e Interval and Tp-e/QTc Ratio in Patients with Hypocalcemia-case Control Study

Abstract Background: To the best of our knowledge, there are studies related to QT and QTc interval in patients with hypocalcemia, but there are no studies evaluating T wave peak and end interval (Tp-e interval), Tp-e/QT and Tp-e/QTc ratios used to evaluate cardiac arrhythmia risk and ventricular repolarization changes rates. Objectives: Therefore, we aimed to investigate whether there is a change in Tp-e interval, Tp-e/QT and Tp-e/QTc ratios in patients with hypocalcemia. Methods: Retrospectively, 29 patients with hypocalcemia in the emergency department were included in the study. Twenty-nine patients with similar age and sex distribution were included in the study as the control group. All patients underwent 12-lead electrocardiography (ECG). In addition to routine measurements, Tp-e interval, Tp-e/QT and Tp-e/QTc ratios were measured on ECG. The study data were grouped as patients with and without hypocalcemia. Results: The mean age of the patients was 66.24 ± 4.95 years. QTc interval, Tp-e interval and Tp-e/QTc values were found to be significantly higher in patients with hypocalcemia (p <0.001 for each). QTc interval, Tp-e interval and Tp-e/QTc ratio showed a significant negative correlation with calcium levels. Conclusion: Tp-e interval and Tp-e/QTc ratios are significantly increased in patients with hypocalcemia compared to those without hypocalcemia and this can be used more effectively in the follow-up of cardiac fatal arrhythmias.

Diagnóstico tardio de síndrome de DiGeorge em criança hipocalcêmica: relato de caso / Late diagnosis of DiGeorge syndrome in hypocalcemic child: case report

A Síndrome de DiGeorge (SDG) decorre de uma microdeleção 22q11.2, sendo considerada uma das microdeleções mais frequentes em humanos. Caracteriza-se por espectro fenotípico bastante amplo, incluindo dificuldade de aprendizado, fácies dismórfica, anomalias cardíacas, hipocalcemia, hipoparatireoidismo, fenda palatina, anomalias do timo, insuficiência imunológica e problemas de fala e alimentação. Contudo, nenhum achado é patognomônico ou mesmo obrigatório. Este relato de caso pretende chamar a atenção para essa síndrome como causa potencial de hipocalcemia e convulsões hipocalcêmicas mesmo após o período neonatal. Reporta-se a história clinico-laboratorial e manejo de um menino de 12 anos, diagnosticado aos sete com SDG em decorrência de facies típica e crise convulsiva hipocalcêmica. O paciente apresentava diagnóstico prévio de transtorno do déficit de atenção e hiperatividade, atraso no desenvolvimento neuropsicomotor e fácies suspeita (micrognatia, orelhas de implantação baixa, hipertelorismo, nariz angular). A hipocalcemia que deflagrou a crise convulsiva foi secundária ao hipoparatireoidismo, sendo tratado com carbonato de cálcio e calcitriol. Houve melhora clínica, porém se manteve hipocalcêmico, apesar de dose otimizada da medicação. O caso é atípico, já que o diagnóstico de SDG foi feito tardiamente, visto que a maioria dos casos é diagnosticada no período neonatal. Além disso, o quadro demonstra a variabilidade de achados clínicos que podem ser encontrados nessa síndrome e a importância de se investigar a SDG em pacientes que apresentem hipocalcemia, mesmo em idades mais avançadas. Salienta-se que o diagnostico tem relevância na implicação dos cuidados à saúde, devido aos riscos imunológicos e cardiológicos apresentados pelos pacientes portadores, devendo ser realizado o mais precocemente possível.(AU)

The DiGeorge Syndrome (DGS) stems from a 22q11.2 microdeletion and is considered one of the most frequent microdeletions in humans. It is characterized by very wide phenotypic spectrum, including learning disability, dysmorphicfacies, cardiac abnormalities, hypocalcemia, hypoparathyroidism, cleft palate, thymus abnormalities, immune impairment and speech and feeding problems. However, any finding is pathognomonic or even mandatory. This case report aims to draw attention to this syndrome as a potential cause of hypocalcemia and hypocalcemic seizures even after the neonatal period. Refers to clinical and laboratory history and management of a boy of 12, diagnosed at 07 with DGS due to typical facies and hypocalcemic seizure. The patient had a previous diagnosis of attention deficit hyperactivity disorder, developmental delay and suspected facies (micrognathia, low-set ears, hypertelorism, angular nose). Hypocalcemia that triggered the seizure was secondary to hypoparathyroidism, being treated with calcium carbon- ate and calcitriol. There was clinical improvement, but hypocalcemic remained despite optimal medication dose. The case is atypical, since the diagnosis DGS was made later, as the majority of cases are diagnosed in the neonatal period. In addition, the table shows the variability of clinical findings that can be found in this syndrome and the importance of investigating the DGS in patients who have hypocalcaemia, even at older ages. Please note that the diagnosis is relevant in the involvement of health care due to immunological and cardiac risks posed by patients and should be done as early as possible.(AU)

Miocardiopatía por hipocalcemia / Hypocalcemic cardiomyopathy

Se presenta el caso de un varón de 56 años, portador de Miocardiopatía dilatada, considerada, en algún momento, como terminal. Se comprobó que presentaba de base una hipocalcemia crónica secundaria a hipoparatiroidismo. Se discute su notable respuesta a administración de calcio e infusión de levosimendan IV fármaco inotropo positivo que actúa sensibilizando al calcio.

osteoporosis in middle-aged and elderly women

To determine knowledge of osteoporosis in middle-aged and elderly Saudi women, and to identify women in need of education on osteoporosis. We conducted this cross-sectional analytical study of 368 women who attended the well-person clinic in a primary health care center [PHCC] in the Dirab area, Riyadh, Saudi Arabia between January and July 2006, using a validated questionnaire. Seventy-six percent of women were postmenopausal, 62% had heard of osteoporosis, and younger women had more knowledge of this disease. The identification of risk factors by the participants ranged from poor to fair. Sixty percent of women identified low calcium intake, lack of exercise was identified as a risk factor in 39%, and 22% identified family history of osteoporosis. Only 48% of the participants could correctly identify calcium rich foods. A considerable number of the Saudi middle-aged and elderly women are unaware of osteoporosis risk factors. Information resources should be easily available for those at high risk

Simultaneous fracture of both femoral necks secondary to a hypocalcemic seizure

We report a rare case of simultaneous fracture of both femoral necks caused by hypocalcemic fit secondary to chronic renal failure. The case was successfully treated by bilateral bipolar arthroplasties

Seizures in the newborn.

Seizures in the newborn period constitute a medical emergency. Subtle seizures are the commonest type of neonatal seizures, other types being clonic, tonic, and myoclonic. Myoclonic seizures carry the worst prognosis in terms of long-term neurodevelopmental outcome. Hypoxic-ischemic encephalopathy is the most common cause of neonatal seizures. Multiple etiologies often co-exist in neonates and hence it is essential to rule out conditions such as hypoglycemia, hypocalcemia, and meningitis before initiating specific therapy. A comprehensive approach for management of neonatal seizures has been described.

A rare cause of heart failure--primary hypoparathyroidism.

We report a patient who presented with congestive heart failure (ejection fraction 24.4%) and who had previous history of convulsions. Our investigations found him to be a case of primary hypoparathyroidism. He showed a dramatic response with the addition of calcium infusion therapy with almost full recovery of left ventricular function (67% ejection fraction after 16 days of the initial echo). We conclude that in a young patient a thorough investigation for heart failure is never complete without looking for endocrine and metabolic causes. The prognosis in these cases is much better, identification and treatment of the same will yield dramatic results.

Miocardiopatía hipoclacémica secundaria a hipoparatiroidismo postiroidectomía: Caso clínico / Hypocalcemic cardiomyopathy secondary to hypoparathyroidism after a thyroidectomy: Report of one case

Calcium plays a central role in ventricular function. We report a 37 year-old woman with chronic hypoparathyroidism and hypocalcemia secondary to a thyroidectomy performed when she was 18 years old, as treatment for a Graves Basedow's disease. She did not have previous cardiac symptoms and rapid progressive congestive heart failure developed after the beginning of levothyroxin supplementation for post operative hypothyroidism. Echocardiography revealed severe systolic and diastolic left ventricular dysfunction, mitral and tricuspid valve insufficiency, systolic pulmonary hypertension, left atrial enlargement and pericardial effusion. Calcium supplementation in addition to diuretics, captopril and digoxin were followed by rapid clinical improvement. Follow up until 18 months showed persistent left ventricular dilatation and systolic dysfunction, with improvement of all the other ecocardiographic findings.

The calcium-sensing receptor and related diseases / O receptor sensor de cálcio e doenças associadas

The calcium-sensing receptor (CASR) adjusts the extracellular calcium set point regulating PTH secretion and renal calcium excretion. The receptor is expressed in several tissues and is also involved in other cellular functions such as proliferation, differentiation and other hormonal secretion. High extracellular calcium levels activate the receptor resulting in modulation of several signaling pathways depending on the target tissues. Mutations in the CASR gene can result in gain or loss of receptor function. Gain of function mutations are associated to Autossomal dominant hypocalcemia and Bartter syndrome type V, while loss of function mutations are associated to Familial hypocalciuric hypercalcemia and Neonatal severe hyperparathyroidism. More than one hundred mutations were described in this gene. In addition to calcium, the receptor also interacts with several ions and polyamines. The CASR is a potential therapeutic target to treatment of diseases including hyperparathyroidism and osteoporosis, since its interaction with pharmacological compounds results in modulation of PTH secretion.

O receptor sensor de cálcio (CASR) ajusta o set point do cálcio extracelular através da regulação da secreção de PTH e da excreção renal de cálcio. O receptor é expresso em diversos tecidos e também está envolvido em outras funções celulares como proliferação, diferenciação e secreção de outros hormônios. Concentrações altas de cálcio extracelular ativam o receptor resultando em modulação de inúmeras vias de sinais intracelulares dependendo do tecido-alvo. Mutações no gene do CASR podem resultar em ganho ou perda de função do receptor. Mutações com ganho de função são associadas à Hipocalcemia autossômica dominante e à Síndrome de Bartter tipo V, enquanto que mutações com perda de função são associadas à Hipercalcemia hipocalciúrica familiar e ao Hiperparatireoidismo neonatal grave. Mais de cem mutações foram descritas neste gene. Além do cálcio, o receptor também interage com inúmeros íons e poliaminas. CASR é um alvo terapêutico potencial para tratamento de doenças incluindo hiperparatireoidismo e osteoporose, pois a sua interação com compostos farmacológicos resulta em modulação da secreção de PTH.

Incidence and etiology of neonatal seizures

To determine the incidence of seizures in admitted neonates and establish the etiological diagnosis. During the study period, 4.8% [97 cases] cases among admitted neonates, having seizures either at presentation or during their stay in the hospital, were included. Hypoxic ischemic encephalopathy was found to be the commonest cause [44.3%] of neonatal seizures, followed by hypoglycemia [19.5%] and hypocalcemia [12.3%] cases. Subtle seizures [41.2%] were the most frequently observed type of seizure, followed by tonic [32.9%] and clonic [20.6%] seizures. Nearly 55% cases had seizures during the first 48 hours of life. Cases of hypoxic ischemic encephalopathy were associated with higher mortality [39.5%] as compared to cases with metabolic seizures. Neonatal seizures were found as a common neurological disorder and presented most commonly as subtle type. Birth asphyxia was the commonest aetiology of neonatal seizures, followed closely by the metabolic seizures

Hipotiroidismo autoimune refratário a altas doses de levotiroxina e hipocalcemia grave / Autoimmune hypothyroidism nonresponsive to high doses of levothyroxine and severe hypocalcemia

Os fatores que dificultam a normalização do TSH no hipotiroidismo merecem atenção especial já que muitos pacientes em reposição de levotiroxina (LT4) encontram-se com TSH fora da faixa normal. Apresentamos uma paciente de 50 anos com quadro de hipotireoidismo autoimune de difícil compensação, associado à anemia, hipocalcemia severa, hipomagnesemia, alterações psíquicas e perda ponderal importante. Após a compensação do hipotiroidismo somente após 325æg/dia de levotiroxina, levantou-se a hipótese de alguma síndrome disabsortiva. Confirmado diagnóstico de doença celíaca através de dosagem de anticorpo anti-gliadina. Com instituição de dieta sem glúten houve melhora dos sintomas relacionados à disabsorção, assim como diminuição da necessidade de levotiroxina para 125æg/dia. Baseando-se em estudos que comprovam a maior prevalência de doença celíaca entre pacientes com desordens autoimunes da tiróide, tem sido proposto por vários autores a realização rotineira de screening para doença celíaca nesse grupo de pacientes.

Predição da etiologia baseada no tipo clínico das convulsoes no período neonatal / Prediction of etiology based on the clinical type of convulsion in the neonatal period

OBJECTIVE: To determine whether the seizure clinical type is predictive of etiology. METHODS: This is a prospective study from 9 neonatal and pediatric intensive care units. Neonatal seizures were defined clinically as described by Volpe. The seizures etiology was defined by positive clinical date, cranial sonogram and laboratory investigation routinely obtained in all neonates with seizures. Correlation between seizures type and etiologies was determined by Odds ration (OR). RESULTS: During one year 89 neonates develop neonatal seizures and the etiologies were determined. The mains seizures types were: subtle (n = 59; 66.3%), tonic (n = 27; 30.4%), clonic (n = 20; 22.4%), myoclonic (n = 4; 4.5%). The most frequent etiologies determined were: asphyxia (n = 60; 67.4%), hypocalcemia (n = 19; 21.3%), hypoglicemia (n = 14; 15.7%), peri-intraventricular hemorrhage (n = 13; 14%). The OR estimate that clonic seizures (OR 5.65 - 1.34 < OR < 23.61%) is predictive to peri-intraventricular hemorrhage. CONCLUSION: The OR estimate high risk to periventricular hemorrhage when the seizure is the clonic type. When the seizure is subtle type the risk is low to this etiology.

Active Absorbable Algal Calcium (AAA Ca): new Japanese technology for osteoporosis and calcium paradox disease.

Active Absorbable Algal Calcium (AAA Ca) is made by submaximally (800 degrees C) heating cleaned oyster shell under reduced pressure and mix it with similarly heated seaweed (Cystophyllum fusiforme). AAA Ca, best absorbed from the intestine among available calcium compounds, consequently most efficiently suppresses parathyroid hormone secretion, increases bone mineral density and decreases vertebral fracture. Aging is associated with calcium deficiency, mostly because of the decreased biosynthesis of 1,25 (OH)2 vitamin D in the kidney. Parathyroid hormone consequently increases, contributing to various diseases associated with aging such as osteoporosis or decrease of calcium in the bone, as well as hypertension, arteriosclerosis, Alzheimer's disease and osteoarthritis due to paradoxical increase of calcium in vascular walls, brain, cartilage and intracellular compartment of many kinds of cells. Mild calcium deficiency is hard to detect despite these serious consequences because of the remarkable constancy of blood calcium concentration maintained by elaborate homeostatic control. Only by successfully counteracting calcium deficiency by AAA Ca with outstanding absorbability, the phenomenon of calcium paradox becomes a recognizable reality within our reach.

Dysphagia after total laryngectomy resulting from hypocalcemia: case report.

Although hypocalcemia is a common postoperative complication of patients who have undergone a total laryngectomy with total thyroidectomy for treatment of laryngeal cancer or cancer of adjacent organs and hypocalcemia can produce the symptom of dysphagia, there has never been a report that hypocalcemia is the cause of dysphagia in these patients. The authors reported two cases who had hypocalcemia after total laryngectomy with total thyroidectomy and presented with sudden and severe dysphagia. However, the symptom of dysphagia was dramatically improved after calcium replacement therapy.

Biochemical abnormalities in neonatal seizures.

OBJECTIVE: The presence of seizure does not constitute a diagnoses but it is a symptom of an underlying central nervous system disorder due to systemic or biochemical disturbances. Biochemical disturbances occur frequently in the neonatal seizures either as an underlying cause or as an associated abnormality. In their presence, it is difficult to control seizure and there is a risk of further brain damage. Early recognition and treatment of biochemical disturbances is essential for optimal management and satisfactory long term outcome. METHODS: The present study was conducted in the department of pediatrics in IGMC Shimla on 59 neonates. Biochemical abnormalities were detected in 29 (49.15%) of cases. RESULT: Primary metabolic abnormalities occurred in 10(16.94%) cases of neonatal seizures, most common being hypocalcaemia followed by hypoglycemia, other metabolic abnormalities include hypomagnesaemia and hyponateremia. Biochemical abnormalities were seen in 19(38.77%) cases of non metabolic seizure in neonates. Associated metabolic abnormalities were observed more often with Hypoxic-ischemic-encephalopathy (11 out of 19) cases and hypoglycemia was most common in this group. CONCLUSION: No infant had hyponateremia, hyperkelemia or low zinc level.