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"apellidos" => "Blanc" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] 2 => array:3 [ "nombre" => "Óscar" "apellidos" => "Segurado-Tostón" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] ] ] 3 => array:3 [ "nombre" => "Pedro" "apellidos" => "García-Cosmes" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] ] ] 4 => array:3 [ "nombre" => "José M." 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It is generally diagnosed during childhood. When the diagnosis is made in adulthood, it may be difficult to distinguish it from secondary hyperparathyroidism,<span class="elsevierStyleSup">2</span> especially in patients with renal failure.</p><p class="elsevierStylePara"><span class="elsevierStyleBold"> </span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">CASE STUDY</span></p><p class="elsevierStylePara"> </p><p class="elsevierStylePara">We report the case of an 18-year-old male with a history of functionally bicuspid aortic valve, who came to the Emergency Department due to colicky hypogastric pain lasting 72 hours, which did not subside when treated with non-steroidal anti-inflammatory drugs (NSAIDs) every 8 hours. The patient had previously experienced similar episodes. On admission, he had blood pressure of 120/80mmHg and I/IV early diastolic murmur, with the rest of the examination being normal. The laboratory test displayed urea 71mg/dl, creatinine 3.69mg/dl, Mg 2.1mg/dl, Ca 5.2mg/dl, P 7.6mg/dl, total protein 6.27g/dl, albumin 3.6g/dl, intact PTH 216pg/ml, 25-hydroxyvitamin D 7.3ng/ml, normal thyroid hormones, venous blood gases: pH 7.37, pO<span class="elsevierStyleInf">2</span> 50mmHg, pCO<span class="elsevierStyleInf">2</span> 37mmHg, bicarbonate 21mmol/l, renal function in urine: EF Na 0.71%, glucose 9mg/dl, urea 946mg/dl, Cr 86.37mg/dl, Na 27mmol/l, K 21.2mmol/l, Ca 7.8mg/day and P 850.2mg/day; systematic and normal urinary sediment, with negative haematuria and proteinuria; blood count: haemoglobin 15.1g/dl, leukocytes 11,000/ul, platelets 167,000/ul. Laboratory tests were reviewed and they revealed previous hypocalcaemia and hyperphosphataemia, with normal renal function, which had not been detected for at least three years (Figure 1). The biochemical study of his parents’ calcaemia, phosphataemia and PTH were normal. The patient’s clinical progression was good after we withdrew the NSAIDs, to which the aetiology of acute renal failure was attributed, oral and intravenous hydration, oral calcium and vitamin D, with kidney function being normal on discharge. Abdominal symptoms subsided after hypocalcaemia was corrected, without recurrences of similar episodes.</p><p class="elsevierStylePara"><span class="elsevierStyleBold"> </span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara"> </p><p class="elsevierStylePara">The term PHP encompasses a heterogeneous group of uncommon metabolic disorders whose common denominator is variable resistance to PTH action.<span class="elsevierStyleSup">1-3</span> Two types of PHP can be distinguished in accordance with the response of urinary cAMP after the intravenous injection of PTH: type I PHP, in which cAMP does not increase following the administration of PTH, and type II PHP, in which cAMP increases.<span class="elsevierStyleSup">4</span> Type I PHP is divided into three subtypes: Ia, Ib and Ic. Patients with subtypes Ia and Ic have an autosomal dominant inheritance pattern and generally present the Albright osteodystrophy phenotype<span class="elsevierStyleSup">4 </span>(short height, obesity, learning difficulties, subcutaneous calcifications and typical skeletal defects) and multi-hormone resistance. Subtype Ib is characterised by isolated resistance to PTH, it may have a normal phenotype or the Albright osteodystrophy phenotype and the defect is usually sporadic, but it is occasionally has autosomal dominant inheritance. In type II PHP, no molecular defect has been identified, but it lacks the Albright phenotype and multi-hormone resistance and it is not usually familial.<span class="elsevierStyleSup">5</span></p><p class="elsevierStylePara">We performed a differential diagnosis between chronic kidney disease, vitamin D deficiency and PHP. The reversibility of renal failure and persistent hypocalcaemia, hyperphosphataemia and elevated PTH ruled out chronic kidney disease as aetiology, and the absence of hypophosphataemia ruled out vitamin D deficiency. We concluded that this patient had type Ib or II PHP, given the absence of the AHO phenotype and multi-hormone resistance. However, we cannot determine clearly whether our patient corresponded to type Ib or type II PHP.</p><p class="elsevierStylePara">In summary, we reported the case of a patient with renal failure, hypocalcaemia, hyperphosphataemia and elevated PTH of long progression. The nephrologists’ knowledge of the calcium-phosphorus metabolism pathophysiology allowed us to make an early diagnosis and carry out early treatment of an uncommon condition for nephrologists and, as its late diagnosis reveals, one that is also uncommon for the other medical specialties. It is necessary to emphasise the importance of early PHP treatment in order to normalise serum calcium and prevent the bone loss that can occur in the long term if a chronically high level of PTH is maintained. The objective of treatment was to normalise serum calcium levels and curb PTH hypersecretion, and as such, vitamin D derivatives and calcium supplements were used.</p><p class="elsevierStylePara"> </p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara"> </p><p class="elsevierStylePara">The authors declare that they have no conflicts of interest related to the contents of this article.</p><p class="elsevierStylePara"><a href="grande/12197_16025_54643_en_f112197.jpg" class="elsevierStyleCrossRefs"><img src="12197_16025_54643_en_f112197.jpg" alt="Progression of calcium and phosphorus (mg/dl) prior to diagnosis and from the start of treatment."></img></a></p><p class="elsevierStylePara">Figure 1. Progression of calcium and phosphorus (mg/dl) prior to diagnosis and from the start of treatment.</p>" "pdfFichero" => "P1-E565-S4505-A12197-EN.pdf" "tienePdf" => true "PalabrasClave" => array:1 [ "en" => array:5 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec439292" "palabras" => array:1 [ 0 => "Pseudohypoparathyroidism" ] ] 1 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec439294" "palabras" => array:1 [ 0 => "Parathyroid hormone" ] ] 2 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec439296" "palabras" => array:1 [ 0 => "Hypocalcaemia" ] ] 3 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec439298" "palabras" => array:1 [ 0 => "Secondary hyperparathyroidism" ] ] 4 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec439300" "palabras" => array:1 [ 0 => "Hyperphosphataemia" ] ] ] ] "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig1" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "12197_16025_54643_en_f112197.jpg" "Alto" => 758 "Ancho" => 1387 "Tamanyo" => 194524 ] ] "descripcion" => array:1 [ "en" => "Progression of calcium and phosphorus (mg/dl) prior to diagnosis and from the start of treatment." ] ] ] "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Garcia Nieto VM, Chahin J. 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2022 January | 83 | 27 | 110 |
2021 December | 53 | 36 | 89 |
2021 November | 51 | 34 | 85 |
2021 October | 65 | 40 | 105 |
2021 September | 69 | 34 | 103 |
2021 August | 74 | 44 | 118 |
2021 July | 50 | 29 | 79 |
2021 June | 43 | 25 | 68 |
2021 May | 96 | 43 | 139 |
2021 April | 127 | 56 | 183 |
2021 March | 76 | 33 | 109 |
2021 February | 74 | 13 | 87 |
2021 January | 54 | 24 | 78 |
2020 December | 56 | 14 | 70 |
2020 November | 43 | 16 | 59 |
2020 October | 43 | 19 | 62 |
2020 September | 26 | 8 | 34 |
2020 August | 57 | 15 | 72 |
2020 July | 47 | 6 | 53 |
2020 June | 47 | 18 | 65 |
2020 May | 62 | 15 | 77 |
2020 April | 57 | 9 | 66 |
2020 March | 44 | 13 | 57 |
2020 February | 51 | 18 | 69 |
2020 January | 62 | 17 | 79 |
2019 December | 57 | 21 | 78 |
2019 November | 53 | 17 | 70 |
2019 October | 36 | 11 | 47 |
2019 September | 53 | 16 | 69 |
2019 August | 68 | 12 | 80 |
2019 July | 62 | 22 | 84 |
2019 June | 44 | 21 | 65 |
2019 May | 47 | 23 | 70 |
2019 April | 100 | 32 | 132 |
2019 March | 69 | 17 | 86 |
2019 February | 53 | 21 | 74 |
2019 January | 51 | 15 | 66 |
2018 December | 110 | 42 | 152 |
2018 November | 139 | 10 | 149 |
2018 October | 115 | 13 | 128 |
2018 September | 93 | 10 | 103 |
2018 August | 58 | 15 | 73 |
2018 July | 56 | 13 | 69 |
2018 June | 63 | 16 | 79 |
2018 May | 68 | 18 | 86 |
2018 April | 68 | 9 | 77 |
2018 March | 57 | 9 | 66 |
2018 February | 44 | 11 | 55 |
2018 January | 43 | 8 | 51 |
2017 December | 50 | 9 | 59 |
2017 November | 50 | 14 | 64 |
2017 October | 43 | 5 | 48 |
2017 September | 58 | 11 | 69 |
2017 August | 76 | 11 | 87 |
2017 July | 55 | 11 | 66 |
2017 June | 66 | 11 | 77 |
2017 May | 63 | 6 | 69 |
2017 April | 64 | 11 | 75 |
2017 March | 59 | 2 | 61 |
2017 February | 56 | 11 | 67 |
2017 January | 38 | 5 | 43 |
2016 December | 61 | 5 | 66 |
2016 November | 69 | 6 | 75 |
2016 October | 122 | 8 | 130 |
2016 September | 139 | 6 | 145 |
2016 August | 189 | 3 | 192 |
2016 July | 217 | 3 | 220 |
2016 June | 126 | 0 | 126 |
2016 May | 140 | 0 | 140 |
2016 April | 115 | 0 | 115 |
2016 March | 108 | 0 | 108 |
2016 February | 109 | 0 | 109 |
2016 January | 108 | 0 | 108 |
2015 December | 130 | 0 | 130 |
2015 November | 72 | 0 | 72 |
2015 October | 109 | 0 | 109 |
2015 September | 89 | 0 | 89 |
2015 August | 95 | 0 | 95 |
2015 July | 80 | 0 | 80 |
2015 June | 50 | 0 | 50 |
2015 May | 61 | 0 | 61 |
2015 April | 8 | 0 | 8 |