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A 10 years old Myanmar boy who had been residing in Malaysia since 2008 had complained of a 3 mo On examination, he was small for age, clubbing, with clinical features suggestive of bronchiectasis. His TB work up were negative. Chest radiograph (CXR) showed bilateral patchy opacities. He was treated as smear –ve PTB with HRZ regime from Jan 2010. He remained symptomatic A 4 yr old Myanmar girl presented 3 wks history of chronic cough with loss of appetite & weight( arrive in Malaysia 9mths earlier, Feb 2011).No contact with anyone with TB. History of taking fresh water crabs . Examination showed child with no clubbing and lung findings suggestive of right pleural effusion . Investigation showed peripheral eosinophilia with CXR (Fig 5) conforming clinical findings. Pleural fluid C/S and AFB C/S negative. Pleural fluid cytology showed presence of Charcot Leyden crystals. No ova seen in pleural fluid/sputum or stool. She too was given a trial of praziquantel with resolution of symptoms on follow-up. PULMONARY PARAGONIMIASIS: IN MYANMAR REFUGEES CHILDREN A 10 year Myanmar boy ,arrived in Malaysia as refugee with his family in 2008.He was referred from district hospital following treatment failure for Pulmonary tuberculosis (PTB). He had chronic cough with loss of weight and low grade fever for 3 months with history of producing rusty brownish sputum. Following chest x-ray(CXR) findings and positive mantoux,he was treated for sputum negative PTB at initial presentation but he had no resolution of symptoms and CXR findings. Physical examination showed a child who was small for his age with clubbing and lung findings of persistent crepitations with occasional wheezing. Extensive investigation with repeat work up for TB and immunodeficiency (1 0 and 2 0 ) did not reveal any significant finding except for eosinophilia of 23% in Full Blood Picture (FBP). Serial CXRs (Figure1a-c) and HRCT thorax (first)were reviewed. Ultrasound guided biopsy of left lung nodule revealed eosinophilic pneumonia. Work-up for endemic mycoses and parasitic infections(sputum and stool) were all negative. Trial of albendezole failed. A repeat CT thorax in 18/2/11s howed bronchiectasis, subpleural masses, cavitating lesions and nodules in both lungs (Figure 2). A right sided open lung biopsy was performed on 23/3/11.The HPE revealed multiple granulomas with central necrosis, dense eosinophilic infiltrations and parasites (adult worm and eggs) typical of pulmonary paragonimiasis. (Figure 3 a and b). The patient remained well except for persistent coughing. His father later admitted to his family eating freshwater crabs whilst in Myanmar. He was discharged whilst awaiting delivery of praziquantel. Patient flew to the USA(end of May 2011) on the day praziquantel was due to be initiated. UNHCR was dutifully notified for commencement of therapy. A 5 yr old Myanmar boy (in Malaysia since July 2011) presented with chronic cough and recurrent skin swelling for 3 months. Associated symptoms of intermittent fever, loss of appetite and weight. No contact with anyone with TB. Investigation while in Rangoon , 2 mths earlier showed massive left pleural effusion with pleural fluid negative for TB.He was started with anti-TB which he defaulted after 2 mths. Physical examination showed a child small for age, no clubbing with lung findings of left massive pleural effusion with shift of trachea. Investigation showed peripheral eosinophilia with CXR confirming presence of massive pleural effusion. Pleural fluid C/S and AFB C/S were negative. No ova seen in pleural fluid &cytology showed eosinophilia. No ova seen in stool/sputum. Parents gave history of fresh water crab ingestion. A trial of praziquantel was given with resolution of symptoms and pleural effusion. Serial CXRs (Fig4a-c) showed significant radiological improvement of the pleural effusion. 1. Dail and Hammer‘s Pulmonary Pathology Vol 1, Non- neoplastic lung disease 2. ImJG, Kong Y, Shin YM, et al. Pulmonary paragonimiasis: clinical & experimental studies. RadioGraphics1993; 13: 575–586 . 3. Paragonimiasis Jung-GI Kee HC, Tropical Medicine Central Resource, chapter 22 4. Paragonimiasis. Jennifer Patterson, Russell W Steele, E- medicine.medscape.com 2009 5. Food-borne trematodiases Jennifer K, Jurg U Clinical Microbiology Reviews July 2009 466-483 CASE REPORT 2 Pulmonary paragonimiasis must be thought of as a differential diagnosis in any person with chronic cough from area of endemicity. It can certainly mimic PTB as both diseases can be endemic in the same geographical areas. Potential patients can be foreigners or locals with a taste for exotic freshwater culinary dishes. Most human paragonimiasis is caused by P. westermani even though other species were found in different geographical location. P. westermani is prevalent in Far East Asia and even South East Asia. The acute phase of infection can be asymptomatic(20%). This is followed by the larval migratory phase from the gastro-intestinal to the pleuro-pulmonary tract: Symptoms may include occasional low grade fever, cough, dyspnoea, chest pain and malaise. The chronic phase of infection sets in about 6 months after initial infection. The main symptom is cough, initially dry, then productive with chocolate or rusty brown sputum. Other symptoms include chest pain/ discomfort, dyspnoea or wheeze. Haemoptysis is seen irregularly and, seldom severe. Other rare signs are of occasional rales, patchy or persistent consolidation, and recurrent pleural effusion. The extra-pulmonary site is cerebral; quite commonly seen in children, constituting about 50% of extra-pulmonary cases. Pulmonary symptoms can precede CNS symptoms in 70% of patients. Do to the varied presentation involving lung and chronicity of symptoms and having the same risk factor of acquiring TB(geographical location),it is NOT surprising that diagnosis of PTB is first entertained in the first 2 cases. Discovery of parogonimiasis in the FIRST CASE was an eye opener. Following this, children who present with similar history with negative work-up for TB & unresponsiveness to standard antibiotics make us think about lung fluke infection. The presence of eosinophilia(peripheral and tissue) with lack of fever and positive history eating raw crustacean help clinch the diagnosis. But eosinophilia only occur in 10-30%; significantly increased with pleurisy. There fore, need to find eggs in sputum, feces, or even pleural fluid to provide evidence. Stool sampling is a better option in children as they tend to swallow sputum. Chest x-ray may be normal in 20% of cases. Migration of juvenile worms may cause pneumonia/ hydrothorax, consolidation or linear opacities. It can even present as a mass/skin infection as in 2nd case.Thin-walled cysts, mass- like consolidation, nodules and bronchiectasis can also been seen in late stages. Our first patient’s CXR first show changes in the migratory phase. Subsequent CXRs and CT thorax depicted the hallmarks of late pulmonary changes and supporting clinical evidence of bronchiectasis. Lung biopsy as in the first case may reveal 1-2 pairs of adult worms encapsulated in cysts surrounded by masses of typical shaped eggs in granulomatous tissue on histology. This finding is classical of the trematode; paragonimus Paragonimiasis is rarely fatal, even without treatment. The drug of choice is Praziquantel. Usual dose is 75mg/kg/day in 3 doses for 3 days. Treatment with Praziquantel has caused patients to cough up live worms but otherwise no other untoward side effects are observed. DISCUSSION Fig. 2: CT Thorax showed a sub pleural mass (*) and cavitating lesion ( ) in right lung base and a nodule on the left ( ). Thahira JM Nazatul HR, Kamarul AMR, Nor Hanim MH,, *Zaleha AM, ** Nik Hashimah NY, ** Zanariah A, Paediatric Department, * Department of Diagnostic Imaging, **Department of Pathology,Paediatric Institute, Hospital Kuala Lumpur, Malaysia Abstract Food borne trematodiases affects about 10% of the world’s population. It is estimated that 20 million people are infected worldwide and more than two hundred and ninety million people are at risk . Paragonimiasis occur in several parts of Far East, West Africa, and Americas. The prevalence of lung fluke as it is commonly known increases in area with numerous human and animal reservoir hosts and abundance availability of first and second intermediate hosts (snails and crab/crayfish respectively) and local custom of eating raw/undercooked food. We described here case series of three Myanmar refugee children with chronic pulmonary symptoms and persistent pulmonary findings requiring a battery of investigations to arrive at the diagnosis. Peripheral eosinophilia with history of ingestion of fresh water crabs and histopathological findings in the FIRST CASE* ;help clinch diagnosis for the following two. The purpose of our presentation is to highlight the clinical presentations, radiological and pathological findings and treatment option in pulmonary paragonimiasis. Keywords: Pulmonary paragonimiasis, refugee, eosinophilia, praziquantel, freshwater crabs . Figure1: (a) CXR 4/12/09 showed linear opacities ( ) and small right pleural effusion( ).(b) Persistent linear opacities and left basal consolidation in CXR 4/1/10.(c) CXR 9/11/10 ; (9 months post anti-TB Rx) showed worsening linear opacities, cavitating lesions ( ) and mass like consolidation () in left mid zone, confirmed on CT Thorax. CASE REPORT 3 CONCLUSION Fig.1(a) Fig.1(c) Fig.1(b) * CASE REPORT 1* F i g 3 a Figure 3: HPE slides showing (a) cross-section of adult worm with thick cuticle and wedge shaped spines. (b) presence of eggs: golden brown, oval shape, slight flattening on one surface Fig 3 a and Fig 3 b Figure 4 a: massive pleural effusion with adjacent collapse of lt lung with bronchiectatic changes Fig 4b-c: showing resolution of effusion. Fig ure 5: Rt pleural effusion with consolidation of Rt lung (pre and post treatment) REFERENCES ACKNOWLEDGEMENT TO HOSPITAL DIRECTOR

PULMONARY PARAGONIMIASIS: IN MYANMAR REFUGEES CHILDREN

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Page 1: PULMONARY PARAGONIMIASIS: IN MYANMAR REFUGEES CHILDREN

A 10 years old Myanmar boy who had been residing in Malaysia since 2008 had complained of a 3 mo On examination, he was small for age, clubbing, with clinical features suggestive of bronchiectasis. His TB work up were negative. Chest radiograph (CXR) showed bilateral patchy opacities. He was treated as smear –ve PTB with HRZ regime from Jan 2010. He remained symptomatic

A 4 yr old Myanmar girl presented 3 wks history of chronic cough with loss of appetite & weight( arrive in Malaysia 9mths earlier, Feb 2011).No contact with anyone with TB. History of taking fresh water crabs .•Examination showed child with no clubbing and lung findings suggestive of right pleural effusion .• Investigation showed peripheral eosinophilia with CXR (Fig 5) conforming clinical findings. Pleural fluid C/S and AFB C/S negative. Pleural fluid cytology showed presence of Charcot Leyden crystals. No ova seen in pleural fluid/sputum or stool.•She too was given a trial of praziquantel with resolution of symptoms on follow-up.

PULMONARY PARAGONIMIASIS: IN MYANMAR REFUGEES CHILDREN

•A 10 year Myanmar boy ,arrived in Malaysia as refugee with his family in 2008.He was referred from district hospital following treatment failure for Pulmonary tuberculosis (PTB). •He had chronic cough with loss of weight and low grade fever for 3 months with history of producing rusty brownish sputum. •Following chest x-ray(CXR) findings and positive mantoux,he was treated for sputum negative PTB at initial presentation but he had no resolution of symptoms and CXR findings.•Physical examination showed a child who was small for his age with clubbing and lung findings of persistent crepitations with occasional wheezing.•Extensive investigation with repeat work up for TB and immunodeficiency (10 and 20) did not reveal any significant finding except for eosinophilia of 23% in Full Blood Picture (FBP).•Serial CXRs (Figure1a-c) and HRCT thorax (first)were reviewed. Ultrasound guided biopsy of left lung nodule revealed eosinophilic pneumonia. Work-up for endemic mycoses and parasitic infections(sputum and stool) were all negative. Trial of albendezole failed.•A repeat CT thorax in 18/2/11s howed bronchiectasis, subpleural masses, cavitating lesions and nodules in both lungs (Figure 2). •A right sided open lung biopsy was performed on 23/3/11.The HPE revealed multiple granulomas with central necrosis, dense eosinophilic infiltrations and parasites (adult worm and eggs) typical of pulmonary paragonimiasis. (Figure 3 a and b).•The patient remained well except for persistent coughing. His father later admitted to his family eating freshwater crabs whilst in Myanmar. He was discharged whilst awaiting delivery of praziquantel. Patient flew to the USA(end of May 2011) on the day praziquantel was due to be initiated. UNHCR was dutifully notified for commencement of therapy.

•A 5 yr old Myanmar boy (in Malaysia since July 2011) presented with chronic cough and recurrent skin swelling for 3 months.•Associated symptoms of intermittent fever, loss of appetite and weight. No contact with anyone with TB.•Investigation while in Rangoon , 2 mths earlier showed massive left pleural effusion with pleural fluid negative for TB.He was started with anti-TB which he defaulted after 2 mths.•Physical examination showed a child small for age, no clubbing with lung findings of left massive pleural effusion with shift of trachea. •Investigation showed peripheral eosinophilia with CXR confirming presence of massive pleural effusion. Pleural fluid C/S and AFB C/S were negative. No ova seen in pleural fluid &cytology showed eosinophilia. No ova seen in stool/sputum.•Parents gave history of fresh water crab ingestion. A trial of praziquantel was given with resolution of symptoms and pleural effusion. •Serial CXRs (Fig4a-c) showed significant radiological improvement of the pleural effusion.

1. Dail and Hammer‘s Pulmonary Pathology Vol 1, Non-neoplastic lung disease2. ImJG, Kong Y, Shin YM, et al. Pulmonary paragonimiasis: clinical & experimental studies. RadioGraphics1993; 13: 575–586.3. Paragonimiasis Jung-GI Kee HC, Tropical Medicine Central Resource, chapter 224. Paragonimiasis. Jennifer Patterson, Russell W Steele, E-medicine.medscape.com 2009 5. Food-borne trematodiases Jennifer K, Jurg U Clinical Microbiology Reviews July 2009 466-483

CASE REPORT 2

Pulmonary paragonimiasis must be thought of as a differential diagnosis in any person with chronic cough from area of endemicity. It can certainly mimic PTB as both diseases can be endemic in the same geographical areas. Potential patients can be foreigners or locals with a taste for exotic freshwater culinary dishes.

• Most human paragonimiasis is caused by P. westermani even though other species were found in different geographical location. P. westermani is prevalent in Far East Asia and even South East Asia.•The acute phase of infection can be asymptomatic(20%). This is followed by the larval migratory phase from the gastro-intestinal to the pleuro-pulmonary tract: Symptoms may include occasional low grade fever, cough, dyspnoea, chest pain and malaise.•The chronic phase of infection sets in about 6 months after initial infection. The main symptom is cough, initially dry, then productive with chocolate or rusty brown sputum. Other symptoms include chest pain/ discomfort, dyspnoea or wheeze. Haemoptysis is seen irregularly and, seldom severe. Other rare signs are of occasional rales, patchy or persistent consolidation, and recurrent pleural effusion. •The extra-pulmonary site is cerebral; quite commonly seen in children, constituting about 50% of extra-pulmonary cases. Pulmonary symptoms can precede CNS symptoms in 70% of patients.•Do to the varied presentation involving lung and chronicity of symptoms and having the same risk factor of acquiring TB(geographical location),it is NOT surprising that diagnosis of PTB is first entertained in the first 2 cases.•Discovery of parogonimiasis in the FIRST CASE was an eye opener. Following this, children who present with similar history with negative work-up for TB & unresponsiveness to standard antibiotics make us think about lung fluke infection. The presence of eosinophilia(peripheral and tissue) with lack of fever and positive history eating raw crustacean help clinch the diagnosis. But eosinophilia only occur in 10-30%; significantly increased with pleurisy. •There fore, need to find eggs in sputum, feces, or even pleural fluid to provide evidence. Stool sampling is a better option in children as they tend to swallow sputum.•Chest x-ray may be normal in 20% of cases. Migration of juvenile worms may cause pneumonia/ hydrothorax, consolidation or linear opacities. It can even present as a mass/skin infection as in 2nd case.Thin-walled cysts, mass-like consolidation, nodules and bronchiectasis can also been seen in late stages.•Our first patient’s CXR first show changes in the migratory phase. Subsequent CXRs and CT thorax depicted the hallmarks of late pulmonary changes and supporting clinical evidence of bronchiectasis. •Lung biopsy as in the first case may reveal 1-2 pairs of adult worms encapsulated in cysts surrounded by masses of typical shaped eggs in granulomatous tissue on histology. This finding is classical of the trematode; paragonimus •Paragonimiasis is rarely fatal, even without treatment. The drug of choice is Praziquantel. Usual dose is 75mg/kg/day in 3 doses for 3 days. Treatment with Praziquantel has caused patients to cough up live worms but otherwise no other untoward side effects are observed.

DISCUSSION

Fig. 2: CT Thorax showed a sub pleural

mass (*) and cavitating lesion (→) in right lung base and a nodule on the left (→).

Thahira JM Nazatul HR, Kamarul AMR, Nor Hanim MH,, *Zaleha AM, ** Nik Hashimah NY, ** Zanariah A, Paediatric Department, * Department of Diagnostic Imaging, **Department of Pathology,Paediatric Institute, Hospital Kuala Lumpur, Malaysia

AbstractFood borne trematodias es affects about 10% of the world’s population. It is es timated that 20 million people are infected worldwide and more than two hundred and ninety million people are at ris k . Paragonimias is occur in s everal parts of Far Eas t, Wes t Africa, and Americas . The prevalence of lung fluke as it is commonly known increas es in area with numerous human and animal res ervoir hos ts and abundance availability of firs t and s econd intermediate hos ts (s nails and crab/crayfis h res pective ly) and local cus tom of eating raw/undercooked food.We des cribed here cas e s eries of three Myanmar refugee children with chronic pulmonary s ymptoms and pers is tent pulmonary findings requiring a battery of inves tigations to arrive at the diagnos is . Peripheral eos inophilia with his tory of inges tion of fres h water crabs and his topathological findings in the FIRST CASE* ;he lp c linch diagnos is for the fo llowing two. The purpos e of our pres entation is to highlight the c linical pres entations , radiological and pathological findings and treatment option in pulmonary paragonimias is . Keywords: Pulmonary paragonimias is , re fugee , eos inophilia, praz iquante l, fres hwater crabs .

Figure1: (a) CXR 4/12/09 showed linear opacities (→) and small right pleural effusion(→).(b) Persistent linear opacities and left basal consolidation in CXR 4/1/10.(c) CXR 9/11/10 ;(9 months post anti-TB Rx) showed worsening linear opacities, cavitating lesions (→) and mass like consolidation (→) in left mid zone, confirmed on CT Thorax.

CASE REPORT 3

CONCLUSION

Fig.1(a) Fig.1(c) Fig.1(b)

*

CASE REPORT 1*

Fig 3a

Figure 3: HPE slides showing (a) cross-section of adult worm with thick cuticle and wedge shaped spines. (b) presence of eggs: golden brown, oval shape, slight flattening on one surface

Fig 3 a and Fig 3 b

Figure 4 a: massive pleural effusion with adjacent collapse of lt lung with bronchiectatic changes Fig 4b-c: showing resolution of effusion.

Fig ure 5: Rt pleural effusion with consolidation of Rt lung (pre and post treatment)

REFERENCES

ACKNOWLEDGEMENT TO HOSPITAL DIRECTOR