Bài giảng A Case Report - Nguyễn Thị Ngọc

A Case ReportBS. Nguyễn Thị Ngọc BS. Nguyễn Thu HươngA 50 day-old maleAddress: Xuân Lam- Ghi Xuân- Hà TĩnhChief complain: Fever, vomitingAdministrationHistory - 10 days before admission: mild fever, vomiting, constipation, no runny nose, no cough, good breastfeeding. - Treatment with oral antibiotic ( Amoxicillin) for 2 days, but his condition wasn’t improved - Admitted to National Hospital of PediatricsPast History	-2nd child, vaginal delivery, full-term, BW 3.4 kg	- His mother: healthy pregnancy, taking prenatal vitamin and mineral supplement	- At 1 month old, he was given vitamin D supple)ment for 3 weeks (stop 1 week before he got sick	- The 1st child died of biliary atresiaOn AdmissionSpontaneous breathing, spo2 98%, good air entry to both sides of lung, no raleRegular heart rate, no murmurAlert, flat fontanelleSoft abdomen, no hepatosplenomegaly.Dehydration (+)Fever: 38,6Initial investigation	CBC: WBC : 14,75 G/L, TT: 38,1%, Hgb: 89g/l, MCV 85,3fl, MCH 30,4pg, TC 341 G/LCRP: 4,03ng/mlBlood electrolytes: Na: 136mmol/l, K: 3,5mmo/l, Clo: 101Leukocytes in urine: ++++Abdominal ultrasound: Nephrocalcinosis.Ultrasound Summary A 50 day-old boy: 10 daysVomiting, mild feverConstipationDehydration Leukocytes in urine: +++Ultrasound: NephrocalcinosisInitial DiagnosisUTI/ NephrocalcinosisInitial treatment: Antibiotic: ceftriaxon	NephrocalcinosisBackgroundThe deposition of calcium in the kidney parenchyma and tubules.Calcium oxalate and calcium phosphate crystals form when the concentration of the reactants exceeds the saturation limit . Nephrocalcinosis is caused by multiple different conditions and clinical presentation depend on the underlying cause.The renal prognosis is determined by the underlying causeClassificationMolecular or chemical: A measurable increase in intracellular calcium concentration exists but is not visible microscopically or via radiographic imaging .Microscopic: Mineral deposits are visible by light microscopic examination of tissue obtained by biopsy but not by radiographic imaging.Macroscopic: Calcification is visible by radiographic imagingCa/creatinin niệu: 4,35 ↑Oxalat niệu: âm tínhUre: 4,3, creatinin: 38Calci: 4,35 ↑; calci ion: 2,05 ↑Điện giải đồ: btPhospho: 1,02pH:7,38. HCO3- : 21 BE: 0,5Calci: 4,35 ↑Calci ion: 2,05 ↑Ca/creatinin niệu: 4,35 ↑ PTH máu: 0,01 ng/l ↓[Vitamin D máu]: 10187,96 mmol/l ↑pH:7,38HCO3- : 21 BE: 0,52 million IU daily for 1 week, 200,000 IU daily for 1.5 weeks, 150,000 IU daily for 2 weeks, 85,000 IU daily for 4 weeks, 50,000 IU daily for 5 weeksTake past history2000 UI/day x 15 days4000 UI/day x 7 daysaaDiagnosisUTI/ Nephrocalcinosis suspected due to vitamin d intoxicationVitamin D metabolismCauses of vitamin d intoxicationExcessive intake of vitamin DSupplementation and food fortificationIatrogenicExcessive production of 1,25[OH]2DLymphoma: Hodgkin and non Hodgkin[MARTIN HEWISON, J Bone Miner Res 2003;18:579–582 . Vitamin D–Mediated Hypercalcemia in Lymphoma: Evidence for Hormone Production by Tumor-Adjacent Macrophages]Granulomatous diseases: sarcoidosis, tuberculosis[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4625306/]Primary hyperparathyroidismMEN (Multiple Endocrine Neoplasia) I & II syndrome: Parathyroid tumorsDecreased degradation of 1,25(OH)2DCYP24A1 loss-of-function mutations, the syndrome of idiopathic infantile hypercalcemia (IIH)Symptoms of vitamin d intoxicationSystemsManifestationsGastrointestinal systemNausea, vomiting, anorexia, diarrhea, constipation, pancreatitisCentral nevous systemLerthagy, weakness, confusion, coma, hypotonic, hyporeflexiaRenal systemPolyuria, nocturia, dehydration, nephrocalcinosis, renal tubularacidosis, renal failureCardiovascular systemHypertension, short QT, arrhythmiasOtherBone pain, muscle and joint pain, painful peri-articular calcinosisDiagnostic cut-offs of levels of serum25[OH]DLaboratory diagnosis25[OH]D level (ng/mL)25[OH]D level (nmol/L)Dificiency 100> 250Intoxication> 150> 325The Endocrine SocietyDiagnosis vitamin D intoxication↑ serum [25OHD] > 375 nmol/LHypercalcemia or hypercalciuria [1,25(OH)2D] normal and ↓PTHHypercalcemia & hypercalciuriaNormal serum [25OHD]↑ 1,25(OH)2D↓ PTH Management of vitamin d intoxicationDiscontinuation of vitamin dRestriction of dietary calcium and vitamin d intakeIV hydration (NS)  loop diuretics (avoid thiazides)Prednisone 1-2mg/kg/d x 4w (prevent renal calcium reabsorption and the production and activity of 1,25(OH)2D, decreasing intestinal calcium absorption)Bisphosphonate: 5-10 mg Alendronate or 0.5–1 mg/kg/dose of PamidronateHemodialysis: acute or chronic renal failure, hypercalcemic crisisProgressionProgressionDateT-Cai-CaCalcuria/Creatininuria9/8/20164,35mmol/l2,05mmol/l4,3522/8/20162,931,5814/9/20162,641,42,612/11/20162,381,222,355/1/20172,251,18ThấpProgession after 2 weeks stop taking vitamind : No feveralertNo vomiting, good brestfeedingConstipation (-)Question Intoxication doseTreatment