COSTARRICENSE DE SEGURO SOCIAL ,(!RTÍCULÓ 1.- Objeto de la ley

(Ref: Nelson 18th, Pg. 1765, O.P. Ghai 7th, Pg. 351)

This is a case of infectious croup (Acute laryngotracheobronchitis) with moderate severity and requires hospitalization and nebulisation with racemic epinephrine.

Laryngo Tracheobronchitis, usually caused by parainfluenza type 1. Bacterial etiology is unusual.

Clinical Features

Usually there is mild cold for a few days before the child develops a brassy cough and inspiratory stridor. The condition may worsen to severe respiratory distress and cyanosis,

Croup is a clinical diagnosis and does not require a radiograph of the neck. However, characteristic "Steeple sign" (Subglottic narrowing) may be seen on PA view of neck.

Severity of Acute LTB

Mild Moderate Severe

General Happy Irritable Agitated or altered

appearance sensorium

Stridor Stridor on coughing Stridor at rest, Stridor at rest and or no stridor gets worse on worsens on agitation

at rest agitation

Respiratory No distress Tacypnea, chest Marked tacypnea with

distress retractions chest retractions

Oxygen >92% in room air >92% in room <92% in room air,

saturation air cyanosis

Management

Mild cases can be managed on ambulatory basis with symptomatic treatment for fever Antibiotics have no role.

Moderately severe patient may need hospitalization and treatment with racemic epinephrine diluted with water.

A single intramuscular dose of dexamethasone (0.3 - 0.6 mg/kg) reduces overall severity during first 24 hrs. More recently inhalation of budesonide (1mg BD) x 2 days has shown good results.

Severe croup may need hospitalization with oxygen inhalation, steroids. Worsening distress may need short term ventilation.

Differential Diagnosis

1. Epiglottitis: H. Influenza type B is the most frequent causative agent.

a. Clinical features: High fever with difficulty in swallowing. He often sits up leaning forwards and saliva dribbling from his chin.

b. Diagnosis: Direct laryngoscopy where epiglottis appears angry red.

c. Management: Hospitalization, Humidified oxygen, fluids, antibiotics (ampicillin/chloramphenicol/ceftriaxone) ± Ventilation.

2. Spasmodic croup: Occurs in children between 1 and 3 yrs. Usually no prodromal symptoms, sudden onset brassy cough and noisy breathing, usually in early hours of morning. Pathogenesis is unknown.

– Treatment: Racemic epinephrine and steroids are helpful.

8. Ans is a. EDTA (Ref: Nelson 19th P. 2450)

The child in question is presenting with signs and symptoms of plumbism as suggested by blue line on gums (Bertonian line), gastrointestinal manifestations like constipation and symptoms of lead encephalopathy (seizures, drowsiness).

As the patient in question in presenting with encephalopathy, he is likely to have blood levels >70 µg/dL and hence, EDTA is the single most important agent for treatment.

Lead Poisoning

Lead is a heavy metal that is purely a toxicant in humans. Lead poisoning may occur in utero, because it readily crosses the placenta from maternal blood. The spectrum of toxicity is similar to that experienced by children after birth.

Major sources of exposure include lead based paints, batteries;

cable sheathing, cosmetic, plastic, toys, mineral supplements.

Metabolism

• After absorption, lead is disseminated throughout the body. Mostly it accumulates in bone and resides for years.

• About 97% in blood is bound on or in Red blood cells.

• The heme pathway is susceptible to lead inhibitory effects.

• Neurotransmitter release is adversely affected by lead.

Clinical Symptoms

1. GIT symptoms: anorexia, abdominal pain, vomiting, constipation 2. CNS symptoms: related to cerebral edema and increased intracranial

pressure.

– Headache, Lethargy, Seizures, Papilledema, Coma.

3. Renal tubular dysfunction at high levels (>100 µg/dL) – Reversible fanconi syndrome.

4. Hemolytic anemia

Diagnosis and Management

Blood lead levels (BLL) remains the gold standard for evaluation. Lead can be measured in urine and hair but has problems of contamination and interpretability.

Drug Treatment

A child with a venous BLL 45 µg/dL or higher should be treated. Children with BLL of 44-70 µg/dL may be treated with a single drug, preferably DMSA.

Those with BLL of 70 µg/dL or greater require 2 drug treatment.

1. EDTA + DMSA / BAL for those without encephalopathy 2. EDTA and BAL for those with encephalopathy

9. Ans. is a. No IgG deposits or C3 deposits on renal biopsy (Ref: Nelson 19th, Pg. 1804 Paediatric Nephrology 5th, Pg. 195) This girl is having generalized edema, grade 3 proteinuria, Fatty + hyaline casts. This is a case of minimal change Nephrotic syndrome.

IgA nephropathy and Alport's syndrome present with acute nephritic syndrome rather than nephrotic syndrome.

Nephritic syndrome is characterized by massive proteinuria (3+ -4+ protein), Hypoalbuminemia (<2.5 gm/dL) Hypercholestrolemia, edema.

Approximately 90% children with Nephrotic syndrome are idiopathic that is nephrotic syndrome. without evidence of a specific systemic cause.

It includes: Minimal change disease (about 85% of total cases)

• Mesangial proliferation

• Focal segmental glomerulo sclerosis

• Membranous nephropathy

• Membranoproliferative glomerulonephritis Differential Diagnosis

• Protein losing enteropathy

• Hepatic failure

• Heart failure

• Protein malnutrition

• Chronic glomenlonephritis Treatment

Children with onset of uncomplicated nephritic syndrome between 1-8 yr of age are likely to have steroid responsive MCNS (Minimal change NS)and steroids therapy may be initiated without a diagnostic renal biopsy. Children with features that make MCNS less likely (Gross hematuria, hypertension, renal insufficiency, hypo-complementenia, age

<1 yr or >8yr) should be considered for renal biopsy before treatment.

About 90% children with MCNS respond to initial steroid therapy.

The initial episode is treated with prednisolone, 2 mg/kg/d for 6 wks.

Thereafter, the dose of prednisolone is reduced to 1.5 mg/kg given on alternate days as a single morning dose for 6 more weeks.

Further prolongation of alternate day prednisolone therapy for 6 months may be beneficial in reducing the risk of subsequent relapses.

10. Ans. is b. Short course of steroids

(Ref: Paediatric Nephrology 5th, Pg. 155-157)

This is a classical case presentation of Henoch - Schonlein purpura with skin, joint, GIT and renal manifestation. Use of oral steroids for 2-3 wks is recommended. Skin biopsy is not mandatory for diagnosis.

Henoch: Schonlein purpura

(Anaphylactoid purpura)

It is the most common systemic small vessel vasculitis in children. It occurs frequently between 3 and 10 yrs.

Diagnosis

Presence of purpura (commonly palpable) or petechiae with lower limb predominance and one of the four following criteria:

1. Abdominal pain 2. Arthritis/ arthralgia

3. Renal: involvement (proteinuria or hematuria or presence of RBC casts)

4. Histopathology showing Leukocytoclastic vasculitis with predominant IgA deposits in skin or proliferative glomerulonephritis with predominant IgA deposits in mesangium on kidney biopsy.

Treatment

1. Joint symptoms only: Rest, analgesia

2. GIT manifestations, orchites, marked subcutaneous edema ' steroids (prednisolone x 2-3 wks)

3. Severe Nephritis or nephritic syndrome, pulmonary hemorrhage 'steroids and cytotoxic drugs.

11. Ans. is b. Oral phosphate and Vit D supplements (Ref: Paediatric Nepthrology 5th, Pg. 324-335) Approach to Refractory Rickets

This case is a classical presentation of hypophosphatemic rickets (normal S. Calcium, low S. Phosphate, Normal S.PTH) and treatment of choice is phosphate and Vit D supplements.

This is clearly a refractory rickets that has not responded to 2 doses of Vit D.

Remember

• S. Phosphate high in chronic renal failure

• S. Parathyroid hormone normal in hypophosphatemic rickets.

Hyphosphosphatemic Rickets

• Most common form of refractory rickets, inherited as a. X-linked dominant: Most common

Mutations in the PHEX gene (chromosome Xp22.1)

This gene is a regulator of FGF - 23 production by osteocytes.

b. Autosomal dominant c. Autosomal recessive

d. Autosomal recessive with hypercalciuria Remember

In hypophosphatemic rickets, symptoms of hypocalcemia (tetany, muscle weakness) are absent.

Dental abnormalities are common in hypophosphatemic x linked rickets.

Treatment

1. Phosphate supplements (Joulie solution: Sodium phosphate, phosphoric acid)

2. Vitamin D supplementation (except hypercalciuric variety) Vit D – Dependent Rickets (VDDR)

• Autosomal recessive, present between 3 - 6 months of age.

1. VDDR type I (Pseudo Vit D deficiency)