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Hypocalcemia is one of the common causes of neonatal seizures and can result in significant morbidity. Among the multitude of etiologies, hypoparathyroidism as a consequence of maternal hyperparathyroidism is an uncommon one. We describe a neonate who presented with hypocalcemic seizures with relative hypoparathyroidism that unmasked a previously undiagnosed and asymptomatic maternal hyperparathyroidism, and explore the difficulties encountered in the management. Despite initial recalcitrance of hypocalcemia to therapy, parathyroid suppression was transient and recovered completely in few months.

Purpose: Late onset neonatal hypocalcemia (LNH) is defined as hypocalcemia detected after day 3 of life. Its occurrence in babies fed with cow’s milk is well understood. Since the advent of modern-day formulas, the incidence has however decreased. Methods: A prospective descriptive study (January 2017 to December 2017) of LNH seizures in neonates was conducted. LNH was defined as the total serum calcium of less than 7 mg/dL in preterm or less than 8 mg/dL in term newborns after 72 h of life. Results: 14 neonates were presented with myoclonic and focal seizures due to late hypocalcemia. All were formula fed. Their mean serum calcium, phosphorus, alkaline phosphatase, magnesium, 25-OH vitamin D, intact PTH levels were 4.93 mg/dL, 9.19 mg/dL, 244 U/L, 1.2 mg/dL, 30 nmol/L, 38.6 pg/mL, respectively. Mean maternal vitamin D levels were 43 nmol/L. Mean hospital stay was 4 days. Clinical response to treatment was brisk in those who were able to shift to total breast feeding early. Conclusions: LNH in formula-fed and vitamin D deficient babies is not uncommon. Emphasis should be laid on exclusive breast feeding even in vitamin D deplete mothers. However, mothers at risk should be supplemented with vitamin D during pregnancy.

Calcium, the most abundant ion in the neonate, plays a role in bone formation, blood coagulation, muscle contractility, and nerve conduction. Calcium levels are regulated via a negative feedback loop by the parathyroid hormone, calcitonin, and vitamin D. Calcium levels in the neonate must be monitored carefully to avoid complications that affect the central nervous system as well as the cardiac and musculoskeletal systems.

Fetus and neonate are dependent maternal supply of calcium for maintaining the calcium profile in physiologic range. The disturbances in maternal calcium homeostasis leads to changes in the baby's calcium. Maternal investigations in neonatal hypocalcemia not only reveal the etiology in the baby but are sometime helpful in unmasking maternal disorder of calcium homeostasis.

Objective: Hyperparathyroidism during pregnancy is associated with increased maternal and neonatal complications. Cinacalcet is a calcimimetic medication that is used in the treatment of hyperparathyroidism; its use in pregnancy is limited to a few case reports.Methods: Case report and literature review.Results: We are reporting on a 37-year-old patient who was admitted to the hospital with dyspnea at 24 weeks gestation. A routine blood test revealed hypercalcemia; corrected calcium level was 3.17 mmol/L, and parathyroid hormone was elevated at 168 pg/mL. The patient was asymptomatic. Following her last delivery 10 years previously, her newborn developed severe tetany and needed treatment with intravenous (IV) calcium. Neck ultrasound was normal. Due to obesity and history of recurrent deep venous thrombosis, neck exploration was not favored. Initial treatment included calcitonin and IV fluids. The aim was to keep her calcium level as normal as possible to avoid any fetal complications. She was started on cinacalcet 15 mg once per day, which was increased later to 15 mg twice daily. By 36 weeks gestation, corrected calcium levels were down to

Infants with neonatal hypocalcemia often present with seizures, and neonatal hypocalcemia can be due to parathyroid (PTH) insufficiency or resistance. Causes of hypocalcemia with PTH elevation include increased phosphate load, vitamin D deficiency (VDD) or defects in metabolism, renal dysfunction, hypomagnesemia, genetic mutations resulting in end-organ resistance to PTH, or critical illness. Hypocalcemia has also been shown to be associated with Gram-negative bacteremia and sepsis in adults. We present the case of a full-term, formula-fed newborn presenting with late-onset hypocalcemic seizures and VDD in the setting of Klebsiella pneumoniae bacteremia. This case highlights that newborns presenting with hypocalcemic seizures should undergo a workup for sepsis.

Introduction: The most prevalent anomalous physical finding during the first week of life is neonatal hyperbilirubinemia (NNH). It is estimated that more than two thirds of newborn babies have acquired clinical jaundice. It was also discovered that in the unconjugated hyperbilirubinemia of most neonates represents a common or exaggerated physiological syndrome. The physical immaturity of infants to handle increased production of bilirubin is referred to as Jaundice. This becomes potentially neurotoxic if severe unconjugated hyperbilirubinemia is left untreated. One of the routine strategies for treating hyperbilirubinemia is known as phototherapy. However it is not a harmless interference. This can produce negative results, such as dehydration, temperature volatility, blood flow redistribution, genotoxicity, rashes on the skin, loose skin, stools, damage to retinas, hypocalcemia and bronze baby syndrome. One of the lesser known but which has a potential adverse effect of phototherapy is hypocalcemia. A total serum calcium concentration of < 7 mg / dl or ionised calcium concentration < 4mg / dl (< 1mol / L) is classified as neonatal hypocalcemia. Hypocalcemia increases the permeability of cells to sodium ions and increases the excitability of cell membranes. Material & Methods: 60 full term neonates were included in present cross sectional study who was admitted to Neonatal Intensive Care Unit (NICU). Full term neonates who completed 37 weeks of gestation with unconjugated hyperbilirubinemia who require phototherapy. Under the normal protocol, traditional phototherapy equipment containing four blue light fluorescent lamps with wavelengths of 410-470 nm was mounted at a distance of 25-35 cm from the skin surface of neonates with entirely covered eyes and genitals. At 15μW / cm2 /nm at the level of the infant's skin, the irradiance during phototherapy was assessed and maintained consistently. Results: Among 60 neonates included in this study there were 27 (45%) male whereas 33 (55%) female neonates. In present study the mean levels of serum calcium before phototherapy was 9.21 with standard deviation of 0.83 while the serum calcium levels after phototherapy were 8.36 with standard deviation of 0.78. There was a statistically significant difference between serum mean calcium levels before and after phototherapy (p=0.001). Conclusion: A major issue is phototherapy induced hypocalcemia. Before beginning and during phototherapy for neonatal jaundice and close monitoring of neonates for signs of hypocalcemia, careful calcium status calculation should be carried out.. After phototherapy, there is a substantial reduction in the amount of serum calcium, but the risk of hypocalcemia in stable full-term neonates is minimal.

Neonatal hypocalcemia (NHC) is one of the most common disorders of calcium metabolism in infants admitted to the NICU. Presentation can range from asymptomatic to generalized seizures or tetany. In this case study, an infant with NHC is presented along with an overview of the pathophysiology, prevalence, diagnosis, and management of NHC for neonatal clinicians.

Context Familial hypocalciuric hypercalcemia type 1 (FHH1) is caused by loss-of-function mutations of the calcium-sensing receptor (CaSR) and is considered a benign condition associated with mild-to-moderate hypercalcemia. However, the children of parents with FHH1 can develop a variety of disorders of calcium homeostasis in infancy. Objective The objective of this work is to characterize the range of calcitropic phenotypes in the children of a mother with FHH1. Methods A 3-generation FHH kindred was assessed by clinical, biochemical, and mutational analysis following informed consent. Results The FHH kindred comprised a hypercalcemic man and his daughter who had hypercalcemia and hypocalciuria, and her 4 children, 2 of whom had asymptomatic hypercalcemia, 1 was normocalcemic, and 1 suffered from transient neonatal hypocalcemia and seizures. The hypocalcemic infant had a serum calcium of 1.57 mmol/L (6.28 mg/dL); normal, 2.0 to 2.8 mmol/L (8.0-11.2 mg/dL) and parathyroid hormone of 2.2 pmol/L; normal 1.0 to 9.3 pmol/L, and required treatment with intravenous calcium gluconate infusions. A novel heterozygous p.Ser448Pro CaSR variant was identified in the hypercalcemic individuals, but not the children with hypocalcemia or normocalcemia. Three-dimensional modeling predicted the p.Ser448Pro variant to disrupt a hydrogen bond interaction within the CaSR extracellular domain. The variant Pro448 CaSR, when expressed in HEK293 cells, significantly impaired CaSR-mediated intracellular calcium mobilization and mitogen-activated protein kinase responses following stimulation with extracellular calcium, thereby demonstrating it to represent a loss-of-function mutation. Conclusions Thus, children of a mother with FHH1 can develop hypercalcemia or transient neonatal hypocalcemia, depending on the underlying inherited CaSR mutation, and require investigations for serum calcium and CaSR mutations in early childhood.

Objectives: To assess the prevalence of hypocalcemia in outpatient clinic neonates and its relation to vitamin D and calcium supplementation. Methods: This cross-sectional analytical study was conducted at the University Teaching Hospital from May to October 2016. Data were collected from 100 neonates by interviewing mothers using a structured questionnaire; which included socio-demographic information, maternal and neonatal history; in addition to investigations of serum calcium total and ionized and serum vitamin D level. Results: The prevalence of hypocalcemia was 76%, late hypocalcemia represent 52% of hypocalcemic neonates. The prevalence of hypovitaminosis D was 38%. Hypocalcemia was found more prevalent among neonates with no history of vitamin D supplementation (98.7%), no history of maternal calcium supplementation (57.9%), while they had a history of neonatal jaundice on phototherapy (46.1%) which increased to 53.8% with late hypocalcemia. Conclusion: Neonatal hypocalcemia is widely prevalent in Fayoum governorate with significant association with a history of neonatal jaundice on phototherapy, not receiving maternal calcium or neonatal vitamin D supplementation. Saudi Med J 2018; Vol. 39 (3): 247-253 doi: 10.15537/smj.2018.3.21679 How to cite this article: Elsary AY, Elgameel AA, Mohammed WS, Zaki OM, Taha SA. Neonatal hypocalcemia and its relation to vitamin D and calcium supplementation. Saudi Med J . 2018 Mar;39(3):247-253. doi: 10.15537/smj.2018.3.21679.

Neonatal hypocalcaemia is defined as serum calcium (S-Ca) < 2.0 mmol/L in fullterm newborns and < 1.75 mmol/L in preterm newborns. Neonatal hypocalcaemia is either early onset (3 days of age). Newborns with hypocalcaemia are often asymptomatic, but may present with hypotonia, apnea, poor feeding, jitteriness, seizures, and cardiac failure. Signs of hypocalcaemia rarely occur unless S-Ca drops below 1.75 mmol/L. Neonatal hypocalcaemia can be a result of hypoparathyroidism (transient or primary), increased serum calcitonin, sepsis, asphyxia, hepatopathy, hypomagnesaemia, high phosphate load, transient hypoparathyroidism, and, rarely, transient neonatal pseudohypoparathyroidism (transient resistance to biological actions of parathyroid hormone [PTH]). We present three boys (two with gestational age 39 weeks, one 36 weeks; none of them with either asphyxia or sepsis) with mild hypotonia, where S-Ca in the range of 1.67-1.9 mmol/L was detected within the first three days of life, together with hyperphosphataemia (serum phosphate [P] 2.5-2.6 mmol/L), normomagnesaemia (serum magnesium [Mg] 0.77-0.88 mmol/L), normal alkaline phosphatase (ALP) activity (2.8-4.5 µkat/L) and high serum PTH (40-51 pg/mL; normal = 5-28). In spite of gradual increase of S-Ca, the elevated serum PTH persisted beyond days 3, 4 and 6 in all three boys, together with normal or low-to-normal S-Ca, high or normal-to-high serum P and no increases in serum ALP. The mothers´ S-Ca, P, Mg, ALP, PTH levels were within normal reference ranges. With regard to laboratory results, the diagnosis of transient neonatal pseudohypoparathyroidism (due to immaturity of PTH-receptors) is highly probable.in these three neonates.

Neonatal hypocalcemia and congenital heart defects has been known as the first clinical manifestation of the chromosome 22q11.2 deletion syndrome (22q11DS). However, because of its wide clinical spectrum, diagnosis of 22q11DS can be delayed in children without classic symptoms. We report the case of a girl with the history of imperforate anus but without neonatal hypocalcemia or major cardiac anomaly, who was diagnosed for 22q11DS at the age of 11 after the onset of overt hypocalcemia. She was born uneventfully from phenotypically normal Korean parents. Imperforate anus and partial cleft palate were found at birth, which were surgically repaired thereafter. There was no history of neonatal hypocalcemia, and karyotyping by GTG banding was normal. At the age of 11, hypocalcemia (serum calcium, 5.0 mg/dL) and decreased parathyroid hormone level (10.8 pg/mL) was noted when she visited our Emergency Department for fever and vomiting. The 22q11DS was suspected because of her mild mental retardation and velopharyngeal insufficiency, and a microdeletion on chromosome 22q11.2 was confirmed by fluorescence in situ hybridization. The 22q11DS should be considered in the differential diagnosis of hypocalcemia at any age because of its wide clinical spectrum.

Introduction The risks of primary hyperparathyroidism (pHPT) to pregnant women and their fetuses are well-reported and appear to increase commensurate with serum calcium. The management strategy of pHPT must be adapted in pregnancy and should reflect the severity of hypercalcaemia. However, no guidelines exist to assist clinicians facing this dilemma. Methods The experience of a high-volume multidisciplinary endocrine surgical service in managing a consecutive series of pregnant women with pHPT referred for parathyroidectomy is presented and compared to a non-pregnant cohort with pHPT. Evidence in the published literature is explored via a review on pHPT and pregnancy outcomes. Results Seventeen pregnant women and 247 age range-matched non-pregnant women with pHPT were referred for surgery over 11 years. Serum calcium was higher in the pregnant cohort 2.89mmol/l vs 2.78mmol/l (p=0.03). Pre-operative localisation with ultrasound succeeded in 8(47%) pregnant women and SestaMIBI in 2/6(33% imaged pre-conception) compared to 84(34%) and 102(42%) controls (NS at 0.36 and 0.59). Parathyroidectomy was performed under general anaesthesia between 12 and 28 weeks gestation, with no adverse pregnancy outcomes resulting. Cure rate was 100% vs 96% in controls. Conclusions pHPT in pregnancy is a threat to mother and child. Medical management may be appropriate in mild disease (serum calcium

Vitamin D deficiency is common globally with a higher prevalence in women, especially during pregnancy. Among the pregnant women, Vitamin D deficiency was reported up to 80% in the Asian group. Vitamin D deficiency was related to a higher risk of maternal complications including preeclampsia, impaired glucose tolerance, and cesarean section rate, and neonatal complications including low birthweight, neonatal hypocalcemia seizure, and impaired skeletal, lung and immune development. There were no data supporting Vitamin D deficiency screening routinely in pregnancy regarding cost-effectiveness or health benefits. The measurement of Vitamin D in the high-risk group of women is necessary. Subsequent supplement with Vitamin D with and without calcium supplement during pregnancy had been statistically significantly reported to decrease the risk of preeclampsia, preterm birth, and low birth body weight. However, due to a lack of studies, the strategies of dietary and nutritional supplement for fetal growth restriction prevention are not statistically effective and are not yet recommended. The present review is to provide an overview of the clinical and the experimental evidence of Vitamin D deficiency-related complication and review of available options for the prevention and management of these complications.

Introduction: Neonatal hypocalcemia is an uncommon condition but clinical presentation can include severe manifestations such as neuromuscular irritability, tetany, seizure or cardiac conduction abnormalities that require prompt intervention to normalize calcium levels. To our knowledge there have been no reports of neonatal hypocalcemia following maternal SARS-CoV2 infection at the time of birth. Here we report a case of transient late-onset neonatal hypocalcemia complicated by maternal SARS-CoV-2 infection at the time of delivery. Clinical Case: A 13-day old full-term appropriate for gestational age female was born to an asymptomatic mother who tested positive for SARS-CoV2 at the time of delivery. Caretakers noticed minor twitching movements in the first few days of life that were progressively worsening. Initial ionized calcium was 0.6 mmol/L, and labs at time of transfer to our hospital were notable for total calcium of 5.5mg/dL(n 8.9–9.9), and ionized calcium of 0.67mmol/L (n 1.12–1.37 mmol/L). Calcium levels improved after she received IV calcium boluses and was started on continuous IV calcium infusion. Initial phosphorus was 8.3 mg/dL (n 3.2–7.4 mg/dL) and magnesium was 1.2 mg/dL (n 1.5–2.2 mg/dL), while intact PTH was inappropriately low at 12.2 pg/mL (n 10–65 pg/mL), and urine calcium to urine creatinine ratio was below the limits of assay detection (n Conclusions: Hypocalcemia in patients with severe COVID-19 is being increasingly reported. There is minimal data on the effect of perinatal SARS-CoV2 infections on neonatal health. While this report does not establish causation, expanding awareness of neonatal abnormalities following maternal SARS-CoV2 infection at delivery will help to recognize causal associations and improve patient care.

Background: Neonatal hypocalcemia is a metabolic abnormality commonly reported. Late onset hypocalcemia usually manifests after 7 days and requires a long-term therapy. Neonatal hypocalcemia due to hypoparathyroidism can manifest as life-threatening seizures or tetany. Initial management is important to prevent any complication due to this condition. However, the initial management to correct hypocalcemia with the administration of calcitriol, high dose intravenous and oral calcium may take longer to achieve because of hypoparathyroidism. Case summary: We report a case of late onset neonatal hypocalcemia in a nine-days-old baby. He was born on the 35-36 week of gestational age by Caesarean section, without asphyxia nor neonatal infection. The baby had a refractory seizure with low serum calcium and magnesium, high phosphate, normal alkaline phosphatase (ALP) and low calcium creatinine urine ratio. His 25-hidroxy vitamin D (25-OHD) total was as low as 12.9 ng/mL. And, he has a low level of parathyroid hormone (PTH). Adequate correction for hypocalcemia and hypomagnesemia failed to maintain calcium and magnesium to their normal value. Oral calcium gluconolactate and carbonate were added after calcitriol supplementation, but seizure still noticed. After magnesium oxide supplementation was given, another seizure had never occurred. A long-term follow-up on vitamin D and calcium level were required. Conclusion: In a hypocalcemic newborn patient, assessment of the magnesium, vitamin D, and PTH status are needed.

PubMed: 28610460 Objective: Vitamin D has many important functions in our body. Especially in intrauterine and early infancy periods, Vitamin D plays a major role in bone development, growth, and the maturation of tissues such as lung and brain. Fetus is dependent on the mother in terms of Vitamin D and maternal Vitamin D deficiency results in a Vitamin D deficient newborn. The purpose of this study was to determine the levels of Vitamin D in newborns and to investigate the association between Vitamin D status of the baby and neonatal hypocalcemia. Method: Vitamin D, calcium, and parathyroid hormone levels of 750 infants, born between 1 January 2014 and 30 June 2015 and followed in Ondokuz Mayıs University Neonatal Intensive Care Unit were analyzed retrospectively. Blood levels of Vitamin D were checked within 3 days after birth. A 25(OH)D3 level of 30 ng/ml) 25(OH)D3 levels; 68 (9%) had mild, 234 (31%) had moderate and 418 (56%) had severe vitamin D deficiency. No correlation was found between Vitamin D levels and gender, mother’s age, gestational week or birth weight. In 79 (17.2%) preterms, neonatal hypocalcemia was observed. Vitamin D levels of the premature infants who had early neonatal hypocalcemia were statistically significantly lower when compared with those who did not have early neonatal hypocalcemia (p =.02). No significant difference was found between the Vitamin D levels of the term infants who had early neonatal hypocalcemia and those who did not (p=.29). No significant difference was found between the Vitamin D levels of the infants who had late neonatal hypocalcemia and those who did not (in preterm p =.27; in term p =.29). Conclusions: Although lack of Vitamin D is preventable and curable, it is an important health problem for newborns in Turkey. In our study, 56% of the infants were found to have severe lack of Vitamin D and lack of Vitamin D was found to be associated with early neonatal hypocalcemia in preterm newborns. However, long-term effects of lack of Vitamin D in infancy are not fully known. In order to be able to prevent neonatal Vitamin D deficiency, 1200 IU/day vitamin D was supplemented to mothers from the 12th gestational week to 6th month of the birth, which was put into effect by the Ministry of Health in 2011, and should be applied by all health workers. © 2017 Informa UK Limited, trading as Taylor & Francis Group.

Background This study investigated the evolution of hypocalcemia with age and its associated risk factors in patients with 22q11.2 deletion syndrome (22qDS) and congenital heart defects. Method A retrospective review of the medical records of 16 22qDS patients (nine female, seven male; age range, 20.5-43 years) was performed. The frequency of hypocalcemia, as well as the clinical course of the disease and the presence of other phenotypes, were investigated. Results Ten of the 16 patients (62.5%) had a history of hypocalcemia. Among the 10 patients, the first onset of hypocalcemia occurred in the following developmental periods: neonatal stage, n = 1; early childhood, n = 3; late childhood, n = 2; adolescence, n = 2; and adulthood, n = 2. Neonatal hypocalcemia was observed in one patient, but it was transient. Two patients had sustained hypocalcemia after cardiac surgery. Hypocalcemia in one patient was transient following cardiac surgery, but it recurred during early adulthood. One patient developed hypocalcemia at 43 years of age. Thymus defect tended to be correlated with history of hypocalcemia (P = 0.09). This condition was often transient (60%) and was triggered by physical stress, such as intracardiac surgery. Conclusion In 22qDS patients, hypocalcemia was often mild and transient during childhood, but it may recur in adulthood. Routine clinical measurement of serum calcium is recommended, particularly in 22qDS patients with thymic defect or in patients who are under physical stress.

Purpose A review of case reports and other published data on fetal and neonatal outcomes associated with maternal use of bisphosphonate medications is presented. Summary Bisphosphonates can persist in the bone matrix for years, even after therapy is discontinued, potentially resulting in fetal bisphosphonate exposure during pregnancy. Adverse effects of bisphosphonates on fetal outcomes have been observed in animal studies, but the bisphosphonate doses administered were much higher than those typically used in clinical practice. A literature search of PubMed (1946–May 2014) and ToxNet identified 15 articles describing the use of bisphosphonate medications by women before and/or during pregnancy (in total, the articles described 65 mother–child pairs); the agents used included alendronate, ibandronate, risedronate, etidronate, pamidronate, tiludronate, and zoledronic acid, with the reported durations of use ranging from one-time treatments to periods of months or years. Adverse outcomes possibly attributable to bisphosphonate use included marginal decreases in gestational age and birth weight and transient neonatal electrolyte abnormalities (e.g., hypocalcemia, hypercalcemia, hyperphosphatemia); however, no long-term health consequences were reported in any infant. Overall, the available published data appear to indicate that maternal bisphosphonate use does not pose a high risk of fetal or neonatal harm. Nonetheless, in cases of known or suspected fetal bisphosphonate exposure, monitoring for neonatal hypocalcemia and associated neuromuscular and cardiac symptoms is advised. Conclusion A limited body of published data suggests that maternal use of bisphosphonates before or during pregnancy does not have serious fetal or neonatal adverse effects.

Infants born from mothers with Gestational diabetes mellitus (GDM) experience several complications, including a higher rate of postnatal hypocalcemia. In this study, we investigated the association between calcium sensing receptor (CaSR) and neonatal hypocalcemia observed in GDM pregnancies.Our study consisted of 58 pregnant women with GDM and 40 healthy women and their neonates. CaSR placental expression was evaluated with immunohistochemistry and Western Blot. Three CaSR single nucleotide polymorphisms, A986S, R990G, Q1011E, were evaluated in neonate's genomic DNA. Serum Ca, P, Mg, 25(OH)D and PTH were measured in cord blood and at 2nd day of life.GDM neonates had lower mean cord blood Ca levels than controls (2.47 ± 0.21 mmol/l vs 2.59 ± 0.13 mmol/l, p = 0.001) while 15.5% developed postnatal hypocalcemia. CaSR expression was lower in GDM than in healthy placentas (p0.001). In the GDM group, reduced CaSR immunostaining in the syncytiotrophoblast (p = 0.042) and extravillous cytotrophoblasts (p = 0.002) was associated with lower Ca cord blood levels. Moreover, the absence of the S allele of the A986S polymorphism was associated with lower serum Ca levels both at birth (AA:2.41 ± 0.23 mmol/l, AS + SS: 2.57 ± 0.12 mmol/l, p = 0.002) and at 2nd day of life (AA:2.05 ± 0.22 mmol/l, AS + SS: 2.20 ± 0.18 mmol/l, p = 0.019).Our results showed that CaSR is under-expressed in GDM compared with healthy placentas and this alteration may be associated with the lower Ca levels measured in cord blood of GDM infants. Placental CaSR seems to exert a local effect in fetal Ca homeostasis, which is dissociated from its contribution to the regulation of Ca homeostasis in postnatal life.

Hypocalcemia is a common endocrinological condition in 22q11.2 deletion syndrome. Neonatal hypocalcemia may affect neurodevelopment. We hypothesized that neonatal hypocalcemia would be associated with rare, more severe forms of intellectual disability in 22q11.2 deletion syndrome.We used a logistic regression model to investigate potential predictors of intellectual disability severity, including neonatal hypocalcemia, neonatal seizures, and complex congenital heart disease, e.g., interrupted aortic arch, in 149 adults with 22q11.2 deletion syndrome. Ten subjects had moderate-to-severe intellectual disability.The model was highly significant (P0.0001), showing neonatal seizures (P = 0.0018) and neonatal hypocalcemia (P = 0.047) to be significant predictors of a more severe level of intellectual disability. Neonatal seizures were significantly associated with neonatal hypocalcemia in the entire sample (P0.0001), regardless of intellectual level. There was no evidence for the association of moderate-to-severe intellectual disability with other factors such as major structural brain malformations in this sample.The results suggest that neonatal seizures may increase the risk for more severe intellectual deficits in 22q11.2 deletion syndrome, likely mediated by neonatal hypocalcemia. Neonatal hypocalcemia often remains unrecognized until the postseizure period, when damage to neurons may already have occurred. These findings support the importance of early recognition and treatment of neonatal hypocalcemia and potentially neonatal screening for 22q11.2 deletions.

Objective: Early onset neonatal hypocalcemia (ENH) occurs within 72 hours after birth and screening is recommended for high-risk infants. This study aimed to analyze the risk factors predictive of ENH and evaluate the effectiveness of intervention for ENH in moderate-to-late preterm infants. Methods: This was a retrospective study, examining moderate-to-late preterm infants at 32-36 weeks of gestational age. Perinatal factors were compared between infants with and without hypocalcemia. Related factors were further analyzed to evaluate the validity of the predictive factors for ENH. The effect of intervention was analyzed by comparing changes in serum calcium levels on day 3 and day 7. Results: A total of 112 infants were enrolled. There were 65 infants in the non-hypocalcemic group and 47 in the hypocalcemic group. Between the two groups, only lower magnesium levels on day 1 were related to ENH with an odds ratio of 0.247 using a cutoff value of 1.7 mmol/L for hypocalcemia (sensitivity 42.6%, specificity 80.0%, P=0.005). No infant with hypocalcemia exhibited clinical seizures. Intervention was performed with oral calcium supplements or low-phosphorus milk feeding in 30 infants. The intervention was more effective in resolving hypocalcemia than no intervention (n=17). Conclusion: Lower magnesium levels on day 1 were related to lower calcium levels and a 4-fold higher risk for hypocalcemia. Moderate-to-late preterm infants with hypomagnesemia should be closely monitored for ENH. Additionally, interventions were found to be effective in restoring calcium levels in ENH.

Objective: Rickets due to vitamin D deficiency is an important cause of the infantile hypocalcemia, but congenital rickets is not so common. Vitamin D deficiency may present with hypocalcemia and sometimes with hyperphosphatemia because of the inadequate response of parathyroid hormone to hypocalcemia during early infancy. We report a case of congenital rickets due to maternal vitamin D deficiency. Case: The patient was admitted with hypocalcemic seizure at 10 days of age. Both the baby and her mother had low 25(OH) vitamin D3 levels. Conclusion: Vitamin D deficiency should be considered primarily in the differential diagnosis of neonatal hypocalcemia, particularly in those areas where maternal vitamin D deficiency is common.

Background : Seizure is a common neurological disorder in neonatal age group. Primary metabolic derangement is one of the common reason behind this convulsion during this period. Among metabolic abnormalities, hypocalcaemia is most common followed by hypoglycemia and hypomagnesaemia. Objectives: The present study was carried out to assess calcium status in the serum of neonates with convulsion, where no obvious cause of convulsion was found. Methodology : This prospective case control study was conducted in the neonatal ward of Dhaka Medical College Hospital over one year from July 2003 to June 2004. A total of 50 neonates (1- 28 days) who had convulsion but no apparent reasons of convulsion had been found out, were enrolled as cases and 50 age, sex body weight matched neonates who had no history camalnia admitted cases of were enrolled as controls. After a quick clinical evaluation and control of convulsion and before giving any specific treatment serum calcium status was measured by colormetric determination (O- CRE-SOL Phahalein complex) method. Neonatal hypocalcemia is considered if serum calcium value less than1.75 mmol/L (7 mg/dl). Results: Among a total of fifty cases, 60% had hypocalcemia, and about 20% of controls also had low calcium level without any manifestation. The mean serum calcium level of cases and controls were 1.62 ± 0.29 and 2.07 ± 0.03 mmol/l respectively (p

A seven-day-old male neonate presented with symptomatic hypocalcemia in the form of generalized seizure activity for three minutes. He arrived at the pediatric emergency department in a postictal state. His clinical examination was unremarkable, but his initial laboratory evaluation revealed marked hypocalcemia and hypomagnesemia. The patient received intravenous boluses of calcium gluconate for correction. The patient had bradycardia during the first calcium gluconate infusion, and on the second infusion, he developed frequent premature ventricular contractions, which progressed into polymorphic ventricular tachycardia. Arrhythmia reverted to sinus rhythm after discontinuation of the calcium gluconate infusion without the need for chemical/electrical cardioversion. Subsequently, two extra doses of intravenous calcium gluconate for persistent hypocalcemia were administered safely. The patient was discharged home successfully in a good general condition after stabilization. The emergence of bradycardia during calcium gluconate infusion should be considered a red flag since it can trigger serious cardiac arrhythmia, especially in the presence of electrolyte abnormalities such as hypocalcemia and hypomagnesemia. We report this case to stress the need for continuous cardiac monitoring of children on calcium gluconate infusion even if proper dose, dilution, and rate of infusion are used, as serious cardiac arrhythmia can be unpredictable and may develop at any time.

Congenital heart defects (CHD) are the most common birth abnormalities and the leading non-infectious cause of mortality in infants. CHD may occur as a single abnormality or as part of a syndrome. Two of the associated syndromes are DiGeorge and Williams-Beuren. DiGeorge (DGS) syndrome is a variant of 22q11.2 microdeletion syndrome and is known as CATCH22 syndrome (cardiac abnormalities, anomalies of the face, thymus hypoplasia, cleft-palate and hypocalcemia). The most associated CHD are conotruncal defects. Williams-Beuren syndrome (WBS) is also a microdeletional syndrome characterised by CHD, facial abnormalities, neonatal hypocalcemia, neurological and behavioural disorders. The most associated CHD are the supravalvular aortic stenosis and supravalvular pulmonary stenosis. FISH (Fluorescence in Situ Hybridization) technique is now the standard investigation for the diagnosis of microdeletional syndromes. This study shows the importance of FISH analysis in patients with syndromic congenital disease.

Vitamin D has an important role in bone-metabolism (and its deficiency can cause preterm osteopenia, craniotabe and rickets), but it has also non-calcitropic functions. In fact, vitamin D deficiency is correlated to chronic kidney disease, respiratory infections, type 1 diabetes, psoriasis, Crohn disease and neonatal hypocalcemia. Because of the vitamin D deficiency is a global problem, its role as a drug is fundamental for the human health in all ages.

Calcium gluconate has been widely used in the management of neonatal hypocalcemia. When extravasation of a calcium gluconate infusion occurs, there may be rapid and marked swelling, erythema, and signs of soft tissue necrosis or infection, with ensuing extensive local calcifi cation, called calcinosis cutis. In most cases, calcinosis cutis following extravasation of calcium gluconate can be treated conservatively without any special treatment. However, it is often diffi cult to make a proper diagnosis because the disease is rare in the orthopedic fi eld, there is a time discrepancy between the clinical symptoms and radiographic fi ndings, and most patients are neonates. There are only two reports of this disease in the orthopedic literature. This report describes a neonate with “calcinosis cutis” following extravasation of calcium gluconate. The patient’s family was informed that the data from this case would be submitted for publication, and they gave their consent.