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Sunday, April 14, 2019

WATER DEPRIVATION TEST

WATER DEPRIVATION TEST:

The aims of this test are:
• To establish the diagnosis of diabetes insipidus (DI).
• To distinguish between nephrogenic and central DI.

Principle:
In a normal individual even after a period of water deprivation, homeostatic mechanisms to maintain serum osmolality and euvolemia within the normal range are activated. Urine output decreases and urine becomes progressively concentrated, but serum sodium (reflecting intravascular volume status) and serum osmolality remain normal.

Patients with DI in whom the renal concentrating ability is impaired can maintain euvolemia only by increasing their water intake. When deprived of water, urine output continues to be high and urine remains dilute. The serum sodium and serum osmolality rise.

Precautions and planning of the water deprivation test:

The test should be performed in a day care where a period of observation for 8–10 hours is possible, or in an in-patient setting
Patients with DI may get rapidly dehydrated on water deprivation; hence resource and expertise for fluid resuscitation should be readily available during the test.

Test procedure: Many protocols for water deprivation test are described. Most protocols entail hourly sampling and can be performed if a heparinized IV cannula is left in place and test results are obtained within 1 hour. Hence a modification of this test may be adopted in practice.

Step 1: Thyroid and adrenal function should be assessed before the water deprivation test.

Water deprivation should be started at midnight. No water or drinks or food should be allowed during the period of testing. Baseline weight is recorded; baseline samples are collected for S. sodium, S. osmolality, urine osmolality
Starting at 9 am, hourly estimations of the above parameters are done. The results are procured as soon as possible

Step 1 stopped at 12 noon (after 12 hours of water deprivation) and Step 2 is started

Step 1 may be terminated at any stage and Step 2 is started if:
Urine osmolality is more than 600 mOsm/L as DI is ruled out
If there is weight loss of more than 5% body weight or there is hemodynamic compromise

If S. osmolality exceeds 300 mOsm/kg at any stage, DI is diagnosed and no further testing is required
Serum arginine vasopressin levels may be collected, if available.

Step 2: Child is allowed some water orally. Desmopressin 5 mcg is administered intranasally or 0.3 mcg is given subcutaneously. Hourly samples are collected for the next 4 hours (Table).

Limitations of Water Deprivation Test:

It cannot be performed in neonates and infants due to higher risk of dehydration during the test. In such situations, estimation of serum and urine osmolality may be a useful guide to diagnosis, coupled with a therapeutic trial of desmopressin when clinical suspicion of DI is high.

Interpretation of the water deprivation test:

S. osmolality (mOsm/kg)
U. osmolality (mOsm/kg)
U. osmolality after dDAVP
Diagnosis
<295
>600
Normal response
>295
<300
>600
CDI
>295
300–600
>600
Partial CDI
>295
<300
Rise <100 above baseline
NDI
CDI: Central diabetes insipidus      NDI: Nephrogenic diabetes insipidus 

Friday, April 12, 2019

PEDIATRICS FCPS MCQS AND TOACS: GI FOREIGN BODIES (FB): When to remove?

PEDIATRICS FCPS MCQS AND TOACS: GI FOREIGN BODIES (FB): When to remove?: GI FOREIGN BODIES (FB): When to remove? §   FB in the esophagus such as button battery, sharp FB and symptomatic FB should be removed ...

PEDIATRICS FCPS MCQS AND TOACS: MESENTERIC LYMPHADENOPATHY: When to investigate?

PEDIATRICS FCPS MCQS AND TOACS: MESENTERIC LYMPHADENOPATHY: When to investigate?: MESENTERIC LYMPHADENOPATHY: When to investigate? 1.       Isolated detection of mesenteric lymph nodes in the absence of signs and sym...

PEDIATRICS FCPS MCQS AND TOACS: Pediatric TOACS:

PEDIATRICS FCPS MCQS AND TOACS: Pediatric TOACS:: A term baby is noted to have a degree of FRONTAL BOSSING, as does his mother. A chest X-ray is performed. Q # 01: Describe th...

GI FOREIGN BODIES (FB): When to remove?


GI FOREIGN BODIES (FB): When to remove?

§  FB in the esophagus such as button battery, sharp FB and symptomatic FB should be removed immediately.
§  Coins and smooth FBs in the esophagus can be removed within 24 hours and it is imperative that underlying esophageal stenosis should be excluded.
§  Most FBs in the stomach, which are small, smooth and round, are passed out naturally and therefore it is preferable to observe and wait for at least a week or even more. FBs retained in the stomach for more than 7 days need endoscopy removal.
§  However, larger objects greater than 2 cm, long greater than 5 cm, sharp objects, needles and button battery in the stomach should be removed endoscopically.
§  Button battery ingestion is a common problem in children and these batteries should be removed as early as possible as leaking button batteries can cause perforation and tissue necrosis.


MESENTERIC LYMPHADENOPATHY: When to investigate?


MESENTERIC LYMPHADENOPATHY: When to investigate?

1.      Isolated detection of mesenteric lymph nodes in the absence of signs and symptoms like anemia, anorexia, weight loss, bowel wall thickening or free fluid in the abdomen does not warrant any specific therapy.
2.      Mesenteric nodes are said to be significant if
§  These are more than 5 in number,
§  more than 14 mm along its long axis and
§  Are matted or present in clusters.
Large, matted nodes or nodes in clusters may warrant further investigation.
3.      If the nodes are small it is preferable to repeat USG after 3–6 months.
4.      Diagnostic laparoscopy is planned if there is an increase in size or               in number of the nodes.

Thursday, April 11, 2019

Pediatric TOACS:

A term baby is noted to have a degree of FRONTAL BOSSING, as does his mother. A chest X-ray is performed.





Q # 01: Describe the abnormalities on the X-Rays chest.
Q # 02: What is the diagnosis?

PEDIATRIC NEPHROLOGY: Basic Approach to Hematuria


APPROACH TO HEMATURIA

We should exclude other causes of red urine without RBCs by urine analysis (Dipstick) which includes:-

A.     Heme positive:
a.      Hemoglobinuria in case of acute hemolytic anemia.
          CBC shows fragmented RBCs & reticulocytosis and Hemoglobin in urine
b.      Myoglobinuria in case of rhabdomyolysis (myositis, crush)
  High serum creatine kinase.

B. Heme negative: Foods e.g. Beet roots, black berries.
Drugs e.g. Rifamipicin, Desferal, Nitrofurantoin.
Urate crystals (red diaper).


History
A.     Glomerulonephritis: sore throat/rashes/body swelling
B.      UTI: fever/frequency/dysuria.
C.      Renal stones: colicky abdominal pain/family history.
D.     Coagulopathy: easy bruising.
E.      Trauma
F.      Family history: hematuria, deafness (Alport’s), sickle cell disease.

Examination
A.     Blood Pressure (use age,sex and height appropriate blood pressure centiles)
B.      Abdomen: palpable masses (polycystic kidneys, tumors, hydronephrosis).
C.      Skin: rashes.
D.     Joints: pain/swelling.

Investigations
A.      It is important to identify serious, treatable, and progressive conditions.
B.      During an acute illness, exclude UTI by urine culture.
C.     Asymptomatic or ‘benign haematuria’ in children without growth failure,        hypertension, oedema, proteinuria, urinary casts, or renal impairment is a      frequent finding.

Localize hematuria:



Glomerular
Extra glomerular
Acute nephritic syndrome
Present
Absent
Color
Cola or tea colored
Bright red
Clots
Absent
May present
RBCS Shape
Dysmorphic (distorted)
Normal
RBCS casts
Present
Absent
Proteinuria
> 30 mg / dL.
< 30 mg / dL.



For Glomerular hemturia:

A.     Hematology
  CBC with differential
B.      Chemistry
  Electrolytes, Ca
  BUN/ Creatinine /Creatinine clearance
  Serum protein/Albumin /Cholesterol
  Urine protein
C.   Immunology
  C3/C4
  ASO/Anti-DNase B
  ANA
  Antineutrophil antibody

Reduced C3 in
-        Post infectious glomerulonephritis
-        Systemic lupus nephritis (and low C4)
-        Nephritis with chronic infection
-        Membrano proliferative glomerulonephritis


D.   Renal Biopsy
Unexplained persistent or recurrent gross hematuria
Lupus nephritis
Glomerulonephritis with:
Nephritic nephrosis
Absent low C3
Unexplained acute renal

For extra glomerular hematuria
Step 1: Urine culture
Step 2: Urine calcium/creatinine ratio, rule out sickle cell anemia, renal/bladder ultrasound
Step 3: Urinalysis: siblings, parents, serum electrolytes, Cr, Ca, if crystalluria, urolithiasis, or nephrocalcinosis: 24-hour urine for Ca, creatinine, uric acid, oxalate and if hydronephrosis/pyelocaliectasis: Cystogram, renal scan.

Treatment:
A.     If obvious cause (e.g. UTI), treat.
B.    If complex diagnosis (impaired renal function, proteinuria, or family history)   refer to paediatric nephrology unit.
C.   If no cause found and normal renal function, BP, and no proteinuria, monitor   until resolves.
D.  If no resolution after 6mths or change in any of above parameters refer to paediatric nephrology unit.

Wednesday, April 10, 2019

PEDIATRIC BONE MARROW FAILURE


BONE MARROW FAILURE:

A.     Tri-lineage failure:
Criteria:
1.       Failure of the 3 cell lines with pancytopenia
2.       No organomegaly or lymphadenopathy.

Causes:
a. Congenital
1. Fanconi anemia
2. Familial aplastic anemia
3. Dyskeratosis congenital; Ectodermal dysplasia with:
Skin pigmentation
Mucous membrane leukoplakia
Nail dystrophy
Others: short stature, cataract, mental retardation

b. Acquired
1. Idiopathic
2. Secondary




B. One cell line failure:

Red Cells (hypoplastic anemia; pure red cell anemia)
a. Congenital
1.       Diamond Blackfan anemia
2.       Congenital dyserythropoietic anemia
3.      Pearson's Syndrome
b. Acquired
1.       Idiopathic: transient erythroblastopenia of childhood
2.       Secondary: to Drugs, Infections, Parvo B19, Malnutrition

White Cells
A.     Schwashman Diamond syndrome: pancreatic insufficiency, metaphyseal dysplasia
B.      Kostmann disease (severe congenital neutropenia)
C.      Reticular dysgenesis

Platelets
A.     Congenital amegakaryocytic thrombocytopenia
B.      TAR syndrome (thrombocytopenia absent radii syndrome)




N.B: Dyserythropoiesis (ineffective erythropoiesis).
A.     Primary (congenital dyserythropoietic anemia).
B.      Secondary dyserythropoiesis:
1.       Megaloblastic anemia (folic acid, or vitamin B12 deficiency).
2.       Thalassemia syndromes.
3.      Sideroblastic anemia.
4.      Paroxysmal nocturnal hemoglobinuria

Tuesday, April 9, 2019

System Hypertension: How will it be investigated?

Investigating SYSTEMIC HYPERTENSION in Children

Confirmation:
§  24-hour Ambulatory Blood Pressure Monitoring (ABPM).
§  Calculate mean daytime BP, and sleep BP over 24 hr.
§  ABPM is useful in the evaluation for:
-        White coat hypertension
-        Risk of hypertensive target organ damage
-        Response to pharmacologic therapy 

Workup for secondary causes:
1. Renal
§  Urine analysis, urine culture, renal function tests.
§  Abdominal ultrasound.
§  Renal Doppler.
§  If blood pressure is not controlled on ≥2 anti-hypertensive drugs, then do pre and post captopril renal scintigraphy and/or CT or MRI angiography
§  If strong suspicion of renovascular hypertension, then perform digital subtraction angiography and/or selective renal vein rennin level

2. Cardiac
§  Chest X-ray
§  Echocardiography (For a cause or effect of hypertension)

3. Endocrinal
§  Electrolytes (potassium & sodium).
§  Night time blood or salivary cortisol level which will be increased in Cushing.

4. Tumors
§  24-hour urine vallynile mandilic acid (VMA); (metabolite of catecholamines) in pheochromocytoma and Neuroblastoma and Abdominal ultrasound, CT, MRI.

Monday, April 8, 2019

PEDIATRICS FCPS MCQS AND TOACS: Tests for rheumatic chorea

PEDIATRICS FCPS MCQS AND TOACS: Tests for rheumatic chorea: Tests for rheumatic chorea Cannot maintain arms extended  spooning &  pronation of hands (Choreic hand). Milk maid’s grip: irregula...

Tests for rheumatic chorea

Tests for rheumatic chorea

  • Cannot maintain arms extended 
  • spooning & pronation of hands (Choreic hand).
  • Milk maid’s grip: irregular contraction & relaxations on squeezing examiner hand
  • Wormian movements of tongue upon protrusion (Darting tongue).
  • Examination of handwriting to evaluate fine motor movements

Sunday, April 7, 2019

Causes of recurrent UTI

Causes of recurrent UTI:

1. Obstructive uropathy:

  • VUR either congenital or secondary to UTI
  • Congenital anomalies e.g. phimosis, double ureters etc
  • Renal calculi
  • Neurogenic bladder

2. Catheters or foreign bodies

3. Constipation; elimination disorders

4. Mal-treatment or inadequate treatment of an acute attack