Children With Chronic Cough And Distension Of Abdomen Biology Essay

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12 year old female child resident of Nepal, presented with cough, progressive breathlessness, loss of appetite, two episodes of hemoptysis and low grade intermittent fever of five years duration and abdominal distension with swelling of feet since one year. Child had not been immunized till date. There was history of taking ATT for 3 months one year back. There was no history of chest pain, syncope, palpitation, jaundice, chronic diarrhea, joint swellings, visual disturbance, skin rash, oral ulcers or exposure to industrial pollutants. There was no history of contact with a case of tuberculosis.

What are the causes of persistent or chronic cough in children?

Respiratory: Bronchial Asthma, Pertussis, Tuberculosis, FB aspiration, Interstitial lung disease, Fungal infection, Cystic fibrosis, Bronchiectasis and Hyperimmunoglobin E syndrome (Job syndrome)

Cardiac: Congenital acyanotic heart disease ( L → R Shunt), CCF

Congenital: Tracheo-oesophageal fistula, Broncho-pulmonary disease, Immobile cilia syndrome, Intralobar bronchopulmonary sequestration

Miscellaneous: GERD, Otitis media, Sinusitis, Histiocytosis, HIV, Psychogenic - Tourette syndrome

What are the causes of hemoptysis in children?

Respiratory: Bronchiectasis, Idiopathic Pulmonary hemosiderosis, Cystic fibrosis, Lung Abscess, GpA-BHS pneumonia, Pertussis, Tuberculosis, Foreign body in Laryngo-tracheal bronchial tree

Cardiac : Mitral stenosis, Pulmonary embolism

Miscellaneous: Hemorrhagic disorders, Goodpasture's Syndrome, SLE, Wegner's granulomatosis

In newborn: Hypoxic LBW babies

Define an Un-immunized child?

A child, who is not, immunized as per UIP guidelines for immunization i.e. not immunized for BCG, DPT, Measles and Polio.

What is the importance of BCG vaccination?

BCG has to be given to all children under five years of age since it protects against severe forms of tuberculosis and Neurotuberculosis.

What are the IAP recommendations for vaccinations in this age?

This child should be immunized with diphtheria, acellular pertussis, tetanus, hepatitis A, hepatitis B and rubella vaccine.

What is contact history?

Any child who lives in a household with an adult suffering from tuberculosis, or has taken ATT in the last 2 years.

What are the possible differential diagnoses based on history so far?

Respiratory: Tuberculosis, Interstitial Lung Disease, Asthma, Sarcoidosis

Cardiac: Acquired heart disease - Rheumatic , Cor pulmonale, Cardiomyopathy

HIV

Examination

On examination she had a BMI of 14.5 Kg m2, BP of 80/60 mm of Hg, pulse rate of 80/min and was afebrile. She had pulsus paradoxus, raised JVP, positive Kussmaul sign, pallor, cervical and axillary lymphadenopathy. On respiratory examination: she had barrel shaped chest; air entry was bilaterally equal, crackles were present bilaterally with few wheeze. Per abdomen revealed massive ascites with fluid thrill. On cardiovascular examination apex beat was not visible, heart sounds were faint and no murmur was heard. Other systems were essentially normal.

Define Pulsus paradoxus and how do we measure it?

It is an exaggeration or accentuation of normal drop in systolic BP seen during inspiration and measured by sphygmomanometer by recording systolic BP at the end of expiration and then during full inspiration. A difference in the systolic blood pressure of more than 10 mm Hg is defined as pulsus paradoxus. It is seen in conditions like pericardial effusion, severe asthma or emphysema, restricted cardiomyopathies, pneumothorax, mediastinal mass, tracheal obstruction and pulmonary embolism. It is a misnomer and there is nothing paradoxical about it.

What is the pathophysiology behind pulsus paradoxus?

There are few mechanisms suggested for pulsus paradoxus:

Dilatation of Pulmonary veins during inspiration causes pooling of blood in lungs leading to decreased left atrial filling.

During inspiration there is increased intra-pericardial pressure as a result of traction from its attachment on surrounding structures, this impairs venous return to heart resulting in decreased stroke volume.

Total Heart volume is fixed. In the competition between two ventricles for a fixed total diastolic volume, increased RV filling on inspiration causes impaired filling of left heart resulting in ↓ LV stroke volume.

What is Kussmaul sign?

It is an increase in the jugular venous pressure rather than normal decrease during inspiration. Neck veins become more prominent with inspiration. It is seen in conditions like constrictive pericarditis, cardiac tamponade, severe right sided failure and right ventricular infarct.

What are the differential diagnoses based on history and examination?

Constrictive pericarditis (? Tubercular)

Interstitial Lung Disease / Childhood Asthma (? Cor pulmonale)

Investigations

Hb - 13.6, ESR-18 mm, LFT, RFT, Electrolytes-normal, HIV- Non reactor, sputum for AFB -ve, Mantoux test -ve, Chest X-ray - patchy consolidation RUZ & LLZ, pleural effusion right, Ascitic fluid: Total protein- 2.3 g/dl with albumin of 1.4 g/dl [high SAAG ascitis], WBC 70/cmm with predominant lymphocytes, LDH-143 (Transudate), DNA PCR for mycobacterium tuberculosis and ADA - negative. QuantiFERON-TB Gold test was negative. 2D ECHO showed effusive constrictive Pericarditis with EF>65%. HRCT chest: bilateral pleural & pericardial effusion, right upper lobe alveolar opacities cardiomegaly, pericardial calcification. Bronchoscopy showed granularity of tracheo-bronchial mucosa with widened carina. Right cervical lymph node biopsy showed a reactive picture. Bone marrow aspirate and biopsy showed normal cellularity with increased eosinophilic precursors. Repeat CT scan after 3 months of ATT showed resolution of consolidation.

What is Quanti FERON-TB Gold test? What is its sensitivity and specificity?

It is an ELISA test that measures the interferon-gamma secreted by T cells in response to tubercular antigens which is a cell-mediated immune response in TB-infected individuals. It is an objective, highly reproducible test that yields a "positive" or "negative" answer with no need for interpretation. Also because it is a blood test, it has no adverse reactions. It has a sensitivity of 87 % and specificity of 97-99 %.

How childhood tuberculosis is different from adult tuberculosis?

Adult tuberculosis

Childhood tuberculosis

Reactivation or Re-infection

Primary Infection occurs for the first time

Lowering of immunity leads to reactivation and locally progressive disease

T cell response dissémination in immuno-compromised

No lymphadenopathy

Well marked lymphadenopathy

Hematogenous spread is uncommon

Lymphatic and hematogenous spread more common - miliary / disseminated TB

Cavitory lesion common

Uncommon

Multi bacillary and highly infectious

Paucibacillary and noninfectious

Main cause of death and disability is pulmonary TB

Main cause of death and disability is non pulmonary TB

Healing of lesion is mainly by fibrosis

Calcification is more common, fibrosis is unusual

What are the indications of steroids in tuberculosis?

Adrenal tuberculosis

Endobronchial tuberculosis

CNS TB - TBM

TB pericarditis

TB laryngitis

Renal tract TB

Massive lymph node enlargement

How do you categorize and treat pediatric tuberculosis as per RNTCP?

Cat

RNTCP category

IAP category

Treatment regimen

Cat 1

New sputum positive

Seriously ill smear negative

Seriously ill extra pulmonary

AFB +ve, PPD, Pleural effusion, CNS, Pericardial, genito-urinary and osteo articular

2 H3R3Z3E3

4 H3R3

Cat 2

Sputum smear positive relapse

Treatment failure

-Do-

2 S3R3H3Z3E3/

1 H3R3Z3E3/

5 H3R3E3

Cat3

Sputum smear negative

Extra pulmonary not seriously ill

Primary complex, Lymph node, unilateral Pleural effusion, Skin TB

2 H3R3Z3

4 H3R3

What is the difference between restrictive cardiomyopathy and constrictive pericarditis?

Feature

Constriction

Restriction

Paradoxical pulse

++

Absent

Pericardial knock

+

_

Equal Rt & Lt side filling pressures

+

Lt 3-5 mm Hg > Rt

Ventricular wall thickness

Normal

Increased

Atrial size

LAE

Bi-atrial enlargement

Septal bounce

Present

Absent

Pericardial thickness

Increased

Normal

Pulm Hypertension

Rare

common

Pulm Arterial systolic pressure > 60 mmHg

No

Common

Pericardium

Calcified

No

Course in hospital

Started on antibiotics, Diuretics, Bronchodilators and supportive therapy

In view of resident of Nepal, symptomatic with chronic cough and non-resolving consolidation, constrictive pericarditis and irregular treatment with ATT in past, she was started on ATT (SEHRZ ) along with Prednisolone 2mg / Kg

After six weeks she became asymptomatic, breathlessness decreased, could walk nearly 1 Km, with no orthopnea or PND and started attending school

After 6 months of ATT, she was taken up for pericardiectomy and had uneventful recovery.

Final diagnosis: Constrictive Pericarditis with Calcification

(Possible etiology - Tubercular)

Commentary

Constrictive pericarditis in children is a diagnostic challenge because of its rare occurrence and uncharacteristic clinical picture. Common etiologies are infective, mainly tubercular, collagenosis, uremia, neoplasias, radiation, trauma and granulomatous diseases. Constrictive pericarditis is not common in children. In 235 pericarditis patients treated at the Toronto Hospital for Sick Children over approximately 30 years, only two developed pericardial thickening and constriction.

Clinical manifestations of constrictive pericarditis result mainly from decreased ventricular filling and impaired myocardial contractility, although systolic function can also be altered to a varying degree. The clinical condition in general restricted to systemic and pulmonary congestion. In about 75% of cases, Beck's triad is found, consisting of little symptomatic evidence of cardiac dysfunction, increased venous pressure and ascites, not proportional to the usually discrete or non apparent edema. Hepatomegaly is intense and non-pulsatile. This fact, coupled with the possible presence of hypoproteinemia, mimics chronic hepatic disease and sometimes primary malnutrition. Pericardial calcification is detected in approximately 50% of cases. Electrocardiographic characteristics are restricted to low voltage of QRS complexes and nonspecific alterations in ventricular repolarization. Echocardiography does not allow an accurate evaluation of the degree of constriction.

The definite treatment to be considered is surgery, to be performed as soon as possible after treating the underlying disorder. As in cases of cardiac outflow obstruction, the use of digitalis, diuretics and vasodilators are contraindicated, because tachycardia, increased venous pressure and peripheral vasoconstriction are compensatory hemodynamic responses.

Suggested reading:

WHO guidelines for National Tuberculosis programme on the management of Tuberculosis in Childhood .2006.

RNTCP training module for medical practitioners. Central TB Division, Directorate General of Health Services, Ministry of Health and Family Welfare, New Delhi 2006.

Consensus statement on childhood Tuberculosis. IAP working group: Indian Pediatr 2010; 47:41-55.

Daniel Bernstein. Diseases of Myocardium and Pericardium. In: Kliegman, Behrman, Jenson editors. 18th ed. Nelson textbook of Pediatric s, Vol 2, 2008; 1963-75.

Demmler GJ: Infectious pericarditis in children' Pediatt Infect Dis J 2006:25:165-166.

6. Keith JD. Constrictive pericarditis. In: Rowe RD, Vlad P. Heart Disease in Infancy and Childhood. 2nd ed. New York: The McMillan Co., 1978: 255-8.

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