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.

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.

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

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.

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

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

Hyperosmolar non-ketotic coma

Criteria to diagnose Hyperosmolar non-ketotic coma (Hyperglycemic hyperosmolar state; HHS)

1. Plasma glucose concentration >33.3 mmol/L (600 mg/dL)
2. Arterial pH >7.30
3. Serum bicarbonate >15 mmol/L
4. Small ketonuria, absent to mild ketonemia
5. Effective serum osmolality >320 mOsm/kg
6. Clinically : Stupor or coma, severe dehydration

Disproportionate Short Stature:

A. With short limbs e.g.

- Achondroplasia

- Rickets

- Osteogenesis imperfecta

B. With short trunk e.g.

- Skeletal dysplasia e.g. Ectodermal dysplasia and Morquio s syndrome

- Fanconi anemia

Diagnostic triad of Wilson Disease: 
- Serum ceruloplasmin (< 20 mg/dL).
- Urinary copper excretion after loading dose of D-penicillamine
- Liver biopsy: hepatic copper accumulation.

Rheumatic Fever: Arthritis Treatment

Treatment of Migratory Arthritis in Rheumatic fever:

1.       Bed rest for 2 weeks

2.       Anti-inflammatory drug: Salicylates

a.      Dose: 50-70 mg/kg/day (max = 6 gram /day) For 3-5 days

b.      Then 50 mg/kg/d for 3 weeks.

c.       Then gradual withdrawal monitored by decline in ESR & CRP

d.      Side effects: Gastritis, GI bleeding, Reye syndrome

3.      Daily examination is vital to pick carditis that can present within 2 weeks of the onset

Pediatric Endocrinology

A 3-year-old girl is brought to the emergency department after her parents had found that she had difficulty in waking this morning. She has been ill with an upper respiratory tract infection for the past 2 days, so has not been eating very well. She did not eat any of her dinner yesterday. Her past medical history is significant for short stature and slow weight gain. She is not on any medication. She is sleepy and difficult to arouse. The physical examination findings are otherwise unremarkable. A finger-stick glucose level is 36 mg/dL (2.0 mmol/L). Blood is drawn at the time of hypoglycemia. She is treated with 1 g/kg of intravenous dextrose, after which she becomes alert and talkative.

Laboratory results are shown:

Laboratory test Result

Blood

Glucose:                            32 mg/dL (1.8 mmol/L)

β-Hydroxybutyric acid:       10 mg/dL (1720 μmol/L) 

                                        (Reference range, fasting<4.2 mg/dL [403 μmol/L])

Fatty acids (nonesterified): 80 mg/dL (2.8 mmol/L) 

                                        (Reference range, 17-42mg/dL [0.60-1.49 mmol/L])

Cortisol:                           8 μg/dL (772 nmol/L) 

                                (Reference range, AM 6.2-19.4 μg/dL [171-535 nmol/L])

Growth hormone:      14 ng/mL (14 μg/L) 

                                (Reference range, > 10 ng/mL [10 μg/L] with stimulation)

Lactate:                    10.8 mg/dL (1.2 mmol/L) (Reference range, 4.5-20 mg/dL [0.5-2.2 mmol/L])

Toxicology screen:                                    Negative

Urine

Urinalysis:               Large amount of ketones present

Organic acids:         Normal

Of the following, the MOST likely diagnosis is

A.     Fatty acid oxidation disorder

B.      Glycogen storage disease

C.      Hyperinsulinism

D.     Ketotic hypoglycemia

E.   Dawn phenomena 

Answer on 27/03/2019