Scenario 1 - Part 2:

The patient had been prescribed Vit D and phosphate supplements with a considerable improvement of his symptoms. One year earlier, the patient was seen at the gastroenterology clinic complaining of chronic diarrhea which has been attributed to the phosphate supplements after thorough investigations to exclude other causes. Unfortunately, CT abdomen accidentally revealed asymptomatic nephrolithiasis. 

For three months, the patient stopped his pills in order to control diarrhea, however, he persistently suffered bony pains and muscle weakness. A combination of oral calcium and potassium phosphate in addition to calcitriol were re-administered with partial control of his symptoms. The patient’s follow-up investigations are shown in table 2

Test

Value

S. Creatinine

0.9 mg/dl

S. corrected Calcium 

9.4 mg/dL

S. Phosphorus

1.7 mg/dL

iPTH

62 pg/mL

25-hydroxy vit-D

39 ng/ml (33-100)

Alk-phosphatase/total 

98 U/L (30-130)

 

D- Interpret the above laboratory investigations.

E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.

F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.

G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?

27 Comments

  • Muhammad Soobadar


    D- low po4-, normal vitamin D

    E – diet can help to replenish phosphate in patient with hypophosphatemia cow milk beans are good source

    F- iv supplementation should be avoided if possible as can cause arrythmia , can cause renal failure due to Ca2+/po42- precipiation

    G burosumab

     

    June 30, 2023 at 11:46 pm   
  • Khaldon Rashed Ahmed Moqbil


    A – low phos and normal vit d
    B- oral supplement and food can improve it in mild type
    c-can use iv in sever type
    d- FGF23 blocker

    May 15, 2023 at 10:15 pm   
  • Amna Kununa


    D- Interpret the above laboratory investigations.
    Persistent symptomatic hypophosphatemia with normalization of Vit D

    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.
    Encourage adding more phosphorus to the diet; help in fixing mild hypophosphatemia and prevent future episodes.

    Foods that are high in phosphorus include:

    • Meat and other proteins, such as chicken, turkey, salmon and organ meat like liver.
    • Fat-free dairy products, such as cottage cheese and yogurt.
    • Nuts and seeds, especially pumpkin and sunflower seeds.

    The average adult consumes 1 gram of phosphorus daily. A quart of cow’s milk provides this amount of phosphorus (1 mg phosphorus/mL).

    Dairy products have an additional advantage of supplying absorbable calcium, which can help avoid the hypocalcemia that may result with more aggressive replacement regimens.

    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.

    • Oral Pi is preferable to intravenous phosphate therapy since intravenous repletion can lead to hyperphosphatemia that may result in serious complications such as hypocalcemia, acute kidney injury, and arrhythmias.
    • If intravenous therapy is necessary in patients with severe symptomatic hypophosphatemia or an inability to take oral therapy.

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    The inhibition of excessive FGF23 actions has been considered to be a novel therapy for these hypophosphatemic diseases. Human MAB for FGF23, burosumab, has been shown to improve biochemical abnormalities, roentgenological signs of rickets, growth, fracture healing and impaired mineralization in patients with XLH.

    May 8, 2023 at 3:02 pm   
  • Rania Mahmoud


    D- Interpret the above laboratory investigations.Improvement of hypo vitamin D and ALP with persistent hypophosphatemia
    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.Dairy products mainly plus meat, beans and fish can replenish phosphate needs.Consumption of vitamin D–supplemented foods should also be encouraged.
    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.Intravenous phosphate is potentially dangerous and produce a variety of adverse effects including hypocalcemia due to binding of calcium, renal failure due to calcium phosphate precipitation in the kidneys, and possibly fatal arrhythmias.
    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?

    • Burosumab is an FGF23 neutralising antibody that focused on the treatment of hypophosphatemic rickets in patients with X-linked hypophosphatemia (XLH).
    • In April 2018, the US FDA approved burosumab for the treatment of XLH in adults and children one year of age and older .
    • Burosumab is well tolerated and leads to sustained correction of serum phosphorus levels, continued healing of fractures and pseudofractures, and sustained improvement in key musculoskeletal impairments.
    April 30, 2023 at 10:04 am   
  • Hagar Ali


    A-improvement of hypo vitamin D and ALP with persistent hypophosphatemia
    B-dairy products mainly plus meat and fish can replenish phosphate needs.
    C-avoid using IV unless sever hypophosphatemia and unable to take oral supplement
    IV can lead to sever hypocalcemia and arrhythmia
    D- Burosumab as ant FGF 23 antibody

    April 30, 2023 at 3:09 am   
  • Ahmed Altalawy


    D- Interpret the above laboratory investigations. Improvement in vit d level and alkaline phosphatase with persistent hypophosphatemia .
    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.The food rich in pi include dairy product, meat,fish, nuts. So we recommend oral intake of pi source for replenish of mild hypophosphatemia.and the daily recommended dose is 1000mg/ day.
    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.Parenteral hypophosphatemia is indicated in severe and symptomatic hypophosphatemia < 1 mg/d.
    In asymptomatic and Pi > 1 oral use is preferred.
    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?burosumab, an anti-FGF23 antibody, was developed as a drug for decreasing excess FGF23 .

    April 29, 2023 at 1:01 pm   
  • Asma Aljaberi


    D- Interpret the above laboratory investigations.
    Hypophosphatemia with normalization of Vit D and ALP with normal Ca, and PTH.

    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.
    Dietary phospahte can provide human being with what he needs in 24 hrs.

    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.
    IV parenteral phosphate should be reserved for high risk patient who has hypophosphatemia. It could be used if the patient is not respoding to oral phosphate or not tolerating oral phosphate. IV parentaeral phosphate has a risk of vascuolar calcification for that it should be avoided in patient with Hx of CVD.

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    Collect 24 urine phosphate and calculate Fractional phosphate excretion.
    If Diagnosis is confirmed, with start Burosumab.

    April 29, 2023 at 12:52 am   
  • Rihab Elidrisi


    Could you interpret the above laboratory investigations?Still having hyphophateamia, with better Vit D level and normalised of ALP

    Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.In patients with hypophosphatemia, dietary encouragement for a high PI diet to overcome renal excretion.
    High Pi diet along with Vit D in the same time.

    Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.Oral phosphate use is better than iv Pi used as the later is associated with a lot of complications like cardiac arrhythmia and hypocalcemia.

    The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend for further or novel therapy In view of the current literature?Burosomab it is anti-FGF23 neutralizing Ab ,it is mainly approved for children with XLH.

    April 28, 2023 at 10:58 pm   
  • Mahmud ISLAM


    a slight improvement in phosphorus, normalization of vitamin D level with 2 times the upper limit PTH. Many patients require a large dose of oral phosphate which is sometimes not available but can be prepared by the pharmacy. Oral is preferred as iv phosphate is available though not widely as potassium phosphate vials. This is impractical and inappropriate for those who require replacement on a daily basis.

    April 28, 2023 at 10:46 pm   
  • Rabab Elrefaey


    D.  Interpret the above laboratory investigations.
    Slight improvement in serum phosphorus level and normalised 25 OH vitamin D and alkaline phosphatase level. Other laboratory investigations did not show abnormalities.

    E.     Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.
    A regular diet generally provides all of the phosphate required for the day and more. For patients with phosphate wasting, high-phosphate diets (including dairy products, meats, and beans) should be encouraged, along with phosphate supplements. Cow’s milk, an excellent and accessible source of phosphate, contains 1 mg (0.032 mmol) of elemental phosphate per milliliter. Consumption of vitamin D–supplemented foods should also be encouraged.

    F.      Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.
    Intravenous phosphate is potentially dangerous since it can precipitate with calcium and produce a variety of adverse effects including hypocalcemia due to binding of calcium, renal failure due to calcium phosphate precipitation in the kidneys, and possibly fatal arrhythmias. So, intravenous phosphate supplementation should be reserved for patients with severe symptomatic hypophosphatemia or inability to take oral phosphate.

    G.    The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    Burosumab is an FGF23 neutralising antibody that has been the subject of several recent clinical trials principally focused on the treatment of hypophosphatemic  rickets in patients with X-linked hypophosphatemia (XLH).

    In April 2018, the US FDA approved burosumab for the treatment of XLH in adults and children one year of age and older .

    Data from previous study demonstrates that in participants with XLH, continued treatment with burosumab is well tolerated and leads to sustained correction of serum phosphorus levels, continued healing of fractures and pseudofractures, and sustained improvement in key musculoskeletal impairments.

    April 28, 2023 at 10:05 pm   
  • Mahmoud Elsheikh


    Interpret the above laboratory investigations.

    • Still hypophosphatemia.
    • Corrected vitD

    Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.
    In mild asymptomatic hypophosphatemia:

    • Oral Pi supplements
    • Diet rich in Pi

    Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia
    Parenteral hypophosphatemia is indicated in severe and symptomatic hypophosphatemia < 1 mg/d.

    In asymptomatic and Pi > 1 oral use is preferred.

    The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend for further or novel therapy In view of the current literature?

    • Burosumab; (crysvita) a FGF23 blocker every 2-4 wks
    April 28, 2023 at 8:44 pm   
  • Asmaa Salih KHUDHUR


    D- Interpret the above laboratory investigations.
    Persistence of hypophosphatemia with improvement in vit D3 and ALP.

    E-Discuss the role of dietary phosphate repletion in patients with hypophosphatemia
    The food rich in pi include dairy product, meat,fish, nuts. So we recommend oral intake of pi source for replenish of mild hypophosphatemia.and the daily recommended dose is 1000mg/ day.

    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.

    In Severe hypophosphatemia (< 1.0 mg/dL )in critically ill, intubated patients should be managed with intravenous replacement therapy (0.08–0.16 mmol/kg) over 2-6 hours
    Moderate hypophosphatemia (1.0–2.5 mg/dL )in intubated patients on a ventilator should be managed with intravenous infusion of pi with fluid (0.08–0.16 mmol/kg) over 2-6 hours
    Moderate hypophosphatemia (1.0–2.5 mg/dL)in nonventilated patients should be managed with oral replacement therapy (1000 mg/d)
    The side effect of iv replacement are hypocalcemia, hyperphosphatemia, renal failure,hypotension , and ECG changes. So The safe dose is 15 mmol over 2 hours.

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    Do genetic study to rule out genetic form hypophosphatemia likeFanconi  syndrome or X-linked hypophosphatimic rickets. In edition to the use on noval drug approved by FDA recently, Burosumab , which is a monoclonal antibody against FGF23, used to reduce phosphaturia.

    April 28, 2023 at 5:04 pm   
  • KAMAL ELGORASHI


    Interpret the above laboratory investigations.

    • Still hypophosphatemia.
    • Corrected vitD and ALP.

    Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.In mild asymptomatic hypophosphatemia, oral Pi supplements and a diet rich in Pi can manage the case.
    Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemiaParenteral hypophosphatemia is indicated in severe and symptomatic hypophosphatemia < 1 mg/d.
    In asymptomatic and Pi > 1 oral use is preferred.

    The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend for further or novel therapy In view of the current literature?

    • Burosumab; anti-FGF23 help in reduce renal phosphate loss
    April 28, 2023 at 10:15 am   
  • Riaan Flooks


    D- Interpret the above laboratory investigations.

    • Normal – renal function, PTH, s-Calcium, Vitamin D and ALP
    • Persistent Hypophosphatemia

    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.

    • Dietary phosphate repletion is contra-indicated in Acute, Severe and Symptomatic Hypophosphatemia
    • In patients who can eat, supplementation with food products such as skimmed milk which has 30mmol/L phosphate should be encouraged. other foodstuffs that can be considered would include red meat, poultry, fish and eggs

    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.

    • Symptoms of hypophosphatemia rarely occur unless the serum phosphate concentration is less than 2 mg/dL (0.64 mmol/L)
    • In patients with hypophosphatemia and a serum phosphate less than 2.0 mg/dL (0.64 mmol/L), we suggest phosphate repletion. Even if these patients are not overtly symptomatic, they may have myopathy and weakness that are not clinically apparent
    • In asymptomatic patients with a serum phosphate less than 2.0 mg/dL (0.64 mmol/L), we give oral phosphate therapy.
    • The treatment of symptomatic patients varies with the severity of the hypophosphatemia. We treat with oral phosphate if the serum phosphate is 1.0 to 1.9 mg/dL (0.32 to 0.63 mmol/L).
    • We treat with intravenous (IV) phosphate if the serum phosphate is less than 1.0 mg/dL (0.32 mmol/L) and switch to oral replacement when the serum phosphate exceeds 1.5 mg/dL (0.48 mmol/L)
    • We stop phosphate repletion when the serum phosphate is greater than or equal to 2.0 mg/dL (0.64 mmol/L) unless there is an indication for chronic therapy such as persistent urinary phosphate wasting.
    • Oral dosing:
    • If the serum phosphate is greater than or equal to 1.5 mg/dL (0.48 mmol/L), 1 mmol/kg of elemental phosphorus (minimum of 40 mmol and a maximum of 80 mmol) can be given in three to four divided doses over a 24-hour period
    • If the serum phosphate is less than 1.5 mg/dL (0.48 mmol/L), 1.3 to 1.4 mmol/kg of elemental phosphorus (up to a maximum of 100 mmol) can be given in three to four divided doses over a 24-hour period
    • IV dosing – IV phosphate is potentially dangerous since it can precipitate with calcium and produce a variety of adverse effects including hypocalcemia due to binding of calcium, kidney failure due to calcium phosphate precipitation in the kidneys, and possibly fatal arrhythmias
    • If the serum phosphate concentration is greater than or equal to 1.25 (0.40 mmol/L), we give 0.08 to 0.24 mmol/kg over six hours (up to a maximum total dose of 30 mmol)
    • If the serum phosphate concentration is less than 1.25 mg/dL (0.40 mmol/L), we give 0.25 to 0.50 mmol/kg over 8 to 12 hours (up to a maximum total dose of 80 mmol)
    • The serum phosphate concentration should be monitored every six hours when intravenous phosphate is given, and the patient should be switched to oral replacement when the serum phosphate concentration reaches 1.5 mg/dL (0.48 mmol/L).

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?

    • In this article listed below, the following was concluded:
    • Phosphorus is one of the most abundant minerals in the human body; it is required to maintain bone integrity and mineralization, in addition to other biological processes. Phosphorus is regulated by parathyroid hormone, 1,25-dihydroxyvitamin D3 [1,25(OH)2D3], and fibroblast growth factor 23 (FGF-23) in a complex set of processes that occur in the gut, skeleton, and kidneys. Different molecular mechanisms – overproduction of FGF-23 by tumors responsible for oncogenic osteomalacia, generation of an FGF-23 mutant that is resistant to cleavage by enzymes, and impaired FGF-23 degradation due to a reduction in or loss of the PHEX gene – can lead to FGF-23-stimulating activity and the consequent waste of urinary phosphate and low levels of 1,25(OH)2D3. Conventional treatment consists of multiple daily doses of oral phosphate salts and vitamin D analogs, which may improve radiographic rickets but do not normalize growth. Complications of the conventional long-term treatment consist of hypercalcemia, hypercalciuria, nephrolithiasis, nephrocalcinosis, impaired renal function, and potentially chronic kidney disease.
    • Recently, burosumab, an antibody against FGF-23, was approved as a novel therapy for children and adults with X-linked hypophosphatemia and patients with tumor-induced osteomalacia. Burosumab showed good performance in different trials in children and adults. It increased and sustained the serum phosphorus levels, decreased the rickets severity and pain scores, and improved mineralization.
    • Arch Endocrinol Metab. 2022; 66(5): 658–665.Published online 2022 Nov 10. doi: 10.20945/2359-3997000000555 New treatments for rare bone diseases: hypophosphatemic rickets/osteomalacia Julia Vieira Oberger Marques, 1 Carolina Aguiar Moreira, 2 and Victoria Zeghbi Cochenski Borba 3
    April 26, 2023 at 2:50 pm   
  • Ahmed Wagih


    D- Interpret the above laboratory investigations.The patient has normal calcium level and adequate vit D level, at the same time he has sever hypophosphatemia and low alkaline phosphatase.
    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.Iv phosphate is potentially harmful as it can combine with serum calcium leading to hypocalcemia, acute kidney injury due precipitation in the kidney and fatal arrythmia.
    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    BUROSUMAB is anti-FGF-23 monoclonal antibody. It can increase expressions of type IIa sodium-phosphate cotransporter and 25-hydroxyvitamin-D-1alpha-hydroxylase and suppress expression of 24-hydroxylase in the kidney

    April 25, 2023 at 9:56 am   
  • Rabab ALaa Eldin keshk Rabab


    D- Interpret the above laboratory investigations.
    Improvement in vit d level and alkaline phosphatase with parsistant hypophosphatemia
    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.
    The phosphorus present in most of food mainly animal source so we prefer the oral route in mild to moderate hypophosphatemia about 1000mg /day
    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.
    Severe hypophosphatemia (< 1.0 mg/dL [0.3 mmol/L]) in critically ill, intubated patients or in those with clinical sequelae of hypophosphatemia (eg, hemolysis) should be managed with intravenous replacement therapy (0.08–0.16 mmol/kg) over 2-6 hours
    Moderate hypophosphatemia (1.0–2.5 mg/dL [0.3–0.8 mmol/L]) in patients on a ventilator should be managed with intravenous replacement therapy (0.08–0.16 mmol/kg) over 2-6 hours
    Moderate hypophosphatemia (1.0–2.5 mg/dL [0.3–0.8 mmol/L]) in nonventilated patients should be managed with oral replacement therapy (1000 mg/d)
    The side effect of iv replacement are hypocalcemia, hyperphosphatemia, renal failure,hypotension , and ECG changes. So The safe dose is 15 mmol over 2 hours
    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    Burosumab, an IgG1 monoclonal antibody that binds excess fibroblast growth factor 23 (FGF23), is approved for treatment of X-linked hypophosphatemia and of FGF23-related hypophosphatemia in tumor-induced osteomalacia associated with phosphaturic mesenchymal tumors

    April 24, 2023 at 5:15 pm   
  • Abdulrahman Almutawakel


    D- Interpret the above laboratory investigations

    • Patient still with low phosphorous while vitamin D normalized that main cause for this hypophosphatemia not low vit D, needs to look for other causes , which in this case mostly related to renal excretion (tubular dysfunction

    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia
    Consider dietary phosphate for those asymptomatic patients who are able to take orally
    Phosphorous rich diets are also protein containing products like meat, fish, dairy products, whole grains ,and nuts.
    food additives also hig phosphorous, also can be obtained from processed food (absorption is 90%), Phosphate from animal protein is better absorbed than plant protein (absorption is ranging from 40 to 80%)
    Oral versus parenteral depends on severity and symptoms as oral can cause diarrhea

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?Genetic study is needed

    April 21, 2023 at 11:06 pm   
  • Israa Hammoodi


    D- Interpret the above laboratory investigations.
    The patient has normal s. Creatinine, calcium, PTH, vitamin D and alkaline phosphotase but low s. Phosphorous

    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.

    Dietary phosphate which is mainly available in diary products, meat and nuts and other foods is useful in mild nutritional hypophosphamia but in other causes of hypophosphamia like renal loss or any causes of moderate to sever defeciency is not useful and inadequate.

    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.

    The oral phosphate preparation mainly with sodium or potassium, the oral preparation used for mild hypophosphamia, caution must be taken when give potassium phosphate to patient with renal impairment but it is useful for patients with renal loss like fanconi syndrome or malnutrition, side effect of oral phosphate is diarrhea
    Parenteral phosphate given when patients have moderate or severe hypophosphamia or when patients cannot tolerate the oral preparation but need monitoring of s. Calcium and phosphorus as might lead to hypocalcimia and hyperphosphatemia

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?

    Do fractional excretion of urinary phosphorous, genetic study to diagnose genetic disorders like fanconi syndrome, x-linked hypophosphametic ricket
    Give AntiFGF23 like bunosumab

    April 21, 2023 at 10:25 pm   
  • Ben Lomatayo


    IV. Interpret the above laboratory investigations.

    • The main finding is persistent moderate hypophosphatemia

    V.Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.

    • Consider it in asymptomatic patient and able to take orally, cow milk is good source for Pi
    • Other Pi rich diets are meat, fish,dairy,whole grains ,and nuts.
    • Inorganic Pi is can be obtained from processed food (absorption is 90%)
    • Phosphate from animal protein is better absorbed than plant protein (absorption is ranging from 40 to 80%)
    • Caution should be exercised in pt with severe malnutrition due risk of reseeding-syndrome

    VI.Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.

    1. Moderate hypophosphatemia (>1 mg/dl): Oral Pi 40 to 80 mmol divided into 4 doses per day
    2. Severe hypophosphatemia (<1 mg/dl): IV Pi 15 mmol over 2 hours but the best protocol in terms of safety and effectiveness is depending on Pi level and pt weight.
    3. Side effects of IV Pi : hypocalcemia, hyperphosphatemia, renal failure,hypotension , and ECG changes. The safe dose is 15 mmol over 2 hours

    V.The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?

    • Recently, in 2018, a new therapeutic strategy for XLHR has been opened with the approval of Burosumab from the Food and Drug Administration and the European Medical Agency. Burosumab is a monoclonal antibody inhibiting FGF23, acting in reducing phosphaturia. Safety and efficacy are optimal with significant improvement in Rickets Severity Score. Until now, no studies are available about the application of Burosumab in ADHR patients.
    April 21, 2023 at 9:10 pm   
  • Alaa Abdel Nasser


    D- Interpret the above laboratory investigations.Normal lab apart from hypophosphatemia
    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.

    • Mild to moderately severe, asymptomatic hypophosphatemia
    • Mild to moderately severe, asymptomatic hypophosphatemia may require oral phosphate replacement; however, correcting factors that led to hypophosphatemia usually is sufficient. Mild hypophosphatemia in DKA and gastrointestinal losses often corrects with resolution of the underlying cause.
    • In patients with minimal symptoms or moderate hypophosphatemia (serum phosphate 1-2 mg/dL), providing oral phosphate replacement may be desirable.
    • It is recommended that oral phosphate replacement be used in patients who are symptomatic and have phosphate levels between 1.0-1.9 mg/dL.
    • For patients who are symptomatic and have a serum phosphate level less than 1.0 mg/dL, IV replacement is recommended, followed by oral replacement once serum phosphate levels reach greater than 1.5 mg/dL.
    • The average adult consumes 1 gram of phosphorus daily. A quart of cow’s milk provides this amount of phosphorus (1 mg phosphorus/mL). Dairy products have an additional advantage of supplying absorbable calcium, which can help avoid the hypocalcemia that may result with more aggressive replacement regimens.
    • Severe/symptomatic hypophosphatemia
    • Patients with symptoms of hypophosphatemia or with serum phosphate levels less than 1 mg/dL require IV phosphate replacement.

    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.Our approach to phosphate repletion takes into account the serum phosphate concentration, the presence or absence of overt symptoms of hypophosphatemia, and whether the patient can take oral therapy. If possible, oral is preferred rather than intravenous phosphate therapy since intravenous repletion can lead to hyperphosphatemia that may result in serious complications such as hypocalcemia, acute kidney injury, and arrhythmias. 
    In asymptomatic patients with a serum phosphate less than 2.0 mg/dL (0.64 mmol/L), we give oral phosphate therapy since many of these patients have myopathy and weakness that are not clinically apparent
    ● The treatment of symptomatic patients varies with the severity of the hypophosphatemia:
    ● We treat with oral phosphate if the serum phosphate is 1.0 to 1.9 mg/dL (0.32 to 0.63 mmol/L)
    • We treat with intravenous phosphate if the serum phosphate is less than 1.0 mg/dL (0.32 mmol/L) and switch to oral replacement when the serum phosphate exceeds 1.5 mg/dL (0.48 mmol/L)

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature? burosumab, an anti-FGF23 antibody, was developed as a drug for decreasing excess FGF23

    April 21, 2023 at 1:58 am   
  • Nour Al Natout


    D.bone parameters AP and vitamin D level normalized. Serum phosphorus is low.
    E.+F The recommended daily phosphate intake is 1250 mg /day in adolescence. But it will surely higher for patient with hypophosphatemia. It is important to orally replace phosphate in case of mild and moderate hypophosphatemia (60mmol divided to 3xday)but in case of severe life threatening hypophosphatemia it should be intravenous.
    G. Fgf23 antagonist burosumab, FDA approved for X Linked hypophosphatemia.

    April 20, 2023 at 10:28 pm   
  • HASSAN ALYAMMAHI


    D- Interpret the above laboratory investigation.
    There is an overall improvement in his clinical and biochemical parameters, with persistent but milder hypophosphatemia

    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.
    Phosphorus can be substituted orally or intravenouslyOral treatments consist of phosphate-containing salts (sodium phosphate or potassium phosphate) and dairy products, as milk contains 1 mg/ml of phosphorus.In general, for oral replacement, 60 mmol of phosphate per day in 3–4 divided doses (to prevent diarrhea) of the preferred preparation, administered over 7–10 days, is sufficient to correct moderate hypophosphatemia.Parenteral administration is recommended for patients with severe hypophosphatemia or those who do not tolerate or respond to oral preparations. Na phosphate is included in IV formulations.In severe hypophosphatemia without overt clinical manifestations, a common dosage regimen is 2.5 mg/kg body weight of phosphate over 6 hours, and 5.0 mg/kg body weight over 6 hours in hypophosphatemic emergencies.

    F- Appraise the use of oral versus parenteral phosphate supplementation
    in patients with moderate to severe hypophosphatemia.Mild to moderate hypophosphatemia (especially that resulting from genetic / renal wasting causes) can be treated with oral phosphate supplementation.IV phosphate is reserved for sever life threatening hypophosphatemia

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    Burosumab, an FGF23 antibody

    April 20, 2023 at 7:04 pm   
  • Ashraf Ahmed Mahmoud


    D- Interpret the above laboratory investigations.hypophosphatemia 1.7 ( 3.4-4.5 mg )
    normal PTH .s.Ca. creatinine,alkaline ph and vit D .

    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.
    Phosphorus can be replaced orally or parenterally
    Oral therapies include phosphate-containing salts (sodium phosphate or potassium phosphate) and dairy products as milk contains 1 mg/ml of phosphorus
    Generally, for oral replacement, 60 mmol of phosphate per day in 3–4 divided doses ( to avoid diarrhea ) of the preparation of choice, given over 7–10 days is sufficient to correct moderate hypophosphatemia.
    parentreral in sever hypophosphatemia or for those who do not tolerate or respond to oral preparations. IV formulations include Na phosphate .
    A common regimen is 2.5 mg/kg body weight of phosphate over 6 hours in severe hypophosphatemia without overt clinical manifestations, and 5.0 mg/kg body weight over 6 hours in hypophosphatemic emergencies.

    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.
    Oral phosphate supplement are useful for the treatment of the genetic disorders of phosphate wasting .
    Parenteral phosphate supplementation is used in life-threatening hypophosphatemia .

    G- The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    Recently, burosumab, a monoclonal blocking antibody to FGF23, has been approved for treatment in children and adults.

    April 19, 2023 at 1:47 pm   
  • Elsayed Ghorab


    The lab revealed
    improvement of lab . result of
    phosphate, vit.D , alk phosphatase . level

    The patient presented again with very low phosphorus level 1.2 mg/dl, what do you recommend further or novel therapy In view of the current literature?
    One of the novel therapy is
    Burosumab is monoclonal antibody that binds excess fibroblast growth factor 23 ( FGF 23)
    this action normalizes phosphorus levels
    improve bone mineralization
    improve rickets in children and help to heal fractures in adults

    April 19, 2023 at 1:38 am   
  • Weam El Nazer


    D- Interpret the above laboratory investigations.severe hypophosphatemia with normal ca, PTH, alkaline phosphatase, and vitamin D
    It explains that severe hypophosphatemia is independent of PTH and Vitamin D

    E- Discuss the role of dietary phosphate repletion in patients with hypophosphatemia.
    Medical care for hypophosphatemia is highly dependent on three factors: cause, severity, and duration. Phosphate distribution varies among patients, so no formula reliably determines the magnitude of the phosphate deficit. The average patient requires 1000–2000 mg (32–64 mmol) of phosphate per day for 7–10 days to replenish the body’s stores.

    F- Appraise the use of oral versus parenteral phosphate supplementation in patients with moderate to severe hypophosphatemia.
    The management of patients with hypophosphatemia can be divided into various subgroups based on the severity of the hypophosphatemia and the need for ventilation, as follows:

    • Severe hypophosphatemia (< 1.0 mg/dL [0.3 mmol/L]) in critically ill, intubated patients or in those with clinical sequelae of hypophosphatemia (eg, hemolysis) should be managed with intravenous replacement therapy (0.08–0.16 mmol/kg) over 2-6 hours
    • Moderate hypophosphatemia (1.0–2.5 mg/dL [0.3–0.8 mmol/L]) in patients on a ventilator should be managed with intravenous replacement therapy (0.08–0.16 mmol/kg) over 2-6 hours
    • Moderate hypophosphatemia (1.0–2.5 mg/dL [0.3–0.8 mmol/L]) in nonventilated patients should be managed with oral replacement therapy (1000 mg/d)
    • Mild hypophosphatemia should be managed with oral replacement therapy (1000 mg/d)

    Oral phosphate supplements, although not curative, are useful for the treatment of the genetic disorders of phosphate wasting and can often normalize phosphate levels and decrease bone pain. Treatment considerations are as follows:

    • The patient’s serum phosphate level, calcium level, bone density, and growth should be monitored frequently to ensure the adequacy of treatment.
    • Oral phosphate supplements are also useful for the treatment of possible oncogenic osteomalacia until the time when the tumor can be identified and surgically removed. Oral phosphate supplements are well tolerated except in high doses, which can produce diarrhea.
    • For very mild hypophosphatemia, increased oral phosphate intake from diet alone may be adequate. Foods that are high in phosphate include dairy items, meats, and beans.

    Parenteral phosphate supplementation is generally reserved for patients who have life-threatening hypophosphatemia or nonfunctional gastrointestinal syndromes. Treatment considerations are as follows:

    • In contrast to oral phosphate supplements, parenteral phosphate administration is more likely to have complications; overly rapid administration can result in hypocalcemia, tetany, and hypotension
    • Other complications that may occur include metastatic calcification, hyperkalemia associated with potassium-containing supplements, volume excess, hypernatremia, metabolic acidosis, and hyperphosphatemia
    • Suggested rates of safe delivery of phosphate range from 1-3 mmol/h. Each milliliter of sodium or potassium phosphate solution has 3 mmol/mL; therefore, this translates to 0.3-1 mL/h. An easy-to-use weight-based regimen involves administering 0.08 mmol/kg (2.5 mg/kg) or 0.16 mmol/kg (5 mg/kg) over 6 hours, depending on the severity of the expected phosphate deficit.
    • The more rapid correction has been found to be safe, but the magnitude of the response can be unpredictable. Serum phosphate and calcium levels should be monitored every 6 hours to ensure the maintenance of normal calcium levels and to prevent overcorrection of phosphate deficiency.

    G- The patient presented again with a very low phosphorus level of 1.2 mg/dl, what do you recommend for further or novel therapy In view of the current literature?

    Recently, a neutralizing antibody for FGF23 (burosumab) has emerged as a promising treatment agent for FGF23-mediated disorders.

    April 18, 2023 at 12:21 pm   

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