Title of Case(s)

Background

Salmonella paratyphi infection causes enteric fever and is a notifiable disease in the UK. Disease is typically milder and of shorter duration than typhoid infection and infection confined to the gastrointestinal tract does not always require treatment. Chronic carriage (over 1 year) is uncommon.

Case Presentation Summary

A 4 year-old boy developed fever and diarrhoea for one week whilst travelling in Bolivia. His symptoms resolved without treatment and he was well on return to the UK. Weeks later, he presented to his GP with mild diarrhoea. Salmonella paratyphi type B was identified on stool cultures and he was treated with oral azithromycin for one week. Subsequent stool cultures remained positive and he was excluded from nursery. Further antibiotic treatment was discussed but due to parental concerns regarding antibiotics, him having no symptoms and most carriage self-resolving, his management continued with strict infection control and active surveillance by Public Health England.

One year later, stool cultures remained positive. Immune function tests were normal but an abdominal ultrasound revealed gallstones. He was treated with one month of ciprofloxacin to sterilise the gallbladder prior to possible cholecystectomy. This was not required as he cleared the infection.

Around this time two children were admitted to hospital unwell with fever and Salmonella paratyphi B bacteraemia. Neither had travelled outside of the UK and both had had significant contact with the index case. Both were treated with intravenous ceftriaxone. One cleared the infection quickly and one required a month of oral ciprofloaxacin.

Subsequent typing confirmed the same strains of S.paratyphi. All other contacts screened were negative.

Key Learning Points

Although uncommon, children may become chronic carriers of Salmonella paratyphi B infection. This poses a significant public health risk. Gallstones may be a risk factor for, or consequence of, chronic infection. Prolonged antibiotic therapy may be required in chronic carriers.
1. Public Health England. Enteric fever (typhoid and paratyphoid) England, Wales and Northern Ireland: 2017.

Title of Case(s)

A rare manifestation of Expanded Dengue syndrome

Background

Dengue fever is endemic in India, however hemophagocytic lymphohistiocytosis (HLH) as a manifesatation of expanded dengue syndrome is relatively rare.

Case Presentation Summary

A 3 year old girl from South India was brought to us with history of fever since 7 days, generalized erythematous rash and vomiting. Examination revealed a febrile child with compensated shock, hepatosplenomgaly and polyserositis.

Investigations showed Hb – 9.2, haematocrit – 28.3%, TC – 10,6000 (N-59,% L – 30.6%), plt – 32,000, CRP – 0.136 (negative), liver enzymes were elevated (SGOT - 244, SGPT -125). Work up for common infectious diseases was positive for Dengue IgM and IgG (ELISA) and negative for malaria, leptospira, typhoid and rickettsial infections (Weil -Felix).

A diagnosis of Dengue fever was established and she was treated with iV fluids according to Dengue protocol. She remained hemodynamically stable, however fever continued beyond day 8. Repeat work up showed elevation of CRP (2.21), anaemia and leucopenia (2700). Antibiotics were empirically started for probable sepsis, however cultures were negative. She was investigated for the cause of persisting fever. LDH (891 IU/L), S. ferritin (997 ng/ml), S. triglycerides (385mg/dl), S. fibrinogen (1.555 g/L) and d-Dimer (1892.1 ng/ml), were all suggestive of haemophagocytic lymphohistiocytosis (HLH). Bone marrow aspiration showed increased histiocytic activity with phagocytosis, confirming the diagnosis of secondary HLH.

She was started on steroids (dexamethasone) following which rapid defervescence and regression of hepatosplenomegaly was noted. She was followed up over 8 weeks, during which she was asymptomatic, all parameters normalized and steroids were tapered and stopped.

Key Learning Points

The diagnosis is challenging because persisting fever, cytopenia, hepatomegaly and deranged liver enzymes are all part of the clinical picture of dengue with sepsis which is a common clinical entity. A high index of suspicion is required for early diagnosis and initiation of treatment which leads to favourable outcomes in secondary HLH.

Title of Case(s)

A neglected disease which destroys faces and lives

Background

Children with malnutrition, poor oral hygiene or suffering from childhood diseases which cause immunosuppression are particularly susceptible to severe infections like Noma (cancrum oris), an oro-facial gangrene with multifactorial aetiology, mainly observed in tropical countries.

Case Presentation Summary

A six year old child presented to an MSF hospital with a lesion on her face which first appeared when she was four, rapidly progressed to an extended ulceration. The patient was previously fit and well and fully immunised. However, she suffered of malnutrition when she was younger due to lack of food availability related to the ongoing conflict. At the time she presented to the hospital she was afebrile, suffering from oral pain with two small dark lesions on the tip of the tongue, complete absence of the soft and hard palate with exposed inferior nasal conchae, absence of the right zygomatic and maxillary bones with right anophthalmia. She was able to swallow and to smile but not to vocalize properly. She received oral antibiotic and antifungal therapy and was referred to another MSF project for surgical reconstruction.
A twenty-two month old child presented in pain with the loss of the entire tip of her nose. The lesion had extended very rapidly over the previous three weeks from a small skin lesion, occurring after recovering from measles. She was initially treated with intravenous antibiotics in another hospital, which were not completed due to the lack of ability to pay for care. She was then referred to the same clinic to receive further treatment.

Key Learning Points

The mortality of Noma is high and the survivors live with often disfiguring facial deformities. It could be prevented by improving the living conditions of people at risk and awareness around it, which is still subject of debate.

Title of Case(s)

Unexpected macrophage inclusion bodies

Background

Visceral leishmaniasis (VL) is an uncommon cause of hemophagocytic lymphohistiocytosis (HLH), yet important due to significant treatment implications. It should be considered, even in non-endemic areas.

Case Presentation Summary

A 2,5 year old Swedish girl presented to our clinic due to fever, fatigue and weight loss. She was found to be pancytopenic (Hb 73, WBC 1,1, ANC 0,8, PLT 129). Her bone marrow was hypocellular, without increased blast count or hemophagocytosis. PCR came back positive for Parvovirus, thought to explain her symptoms. She was dismissed with a close follow up.

A month later her symptoms persisted and she had developed abdominal discomfort. Hepatosplenomegaly was noted on ultrasound. She was still pancytopenic. Extended labs revealed hyperferritinemia (16,000 ug/L) and hypertriglyceridemia (9,3 mmol/L). She was diagnosed with HLH, and was started on dexamethasone, etoposide and cyclosporine as per the 2004-HLH protocol. Another bone marrow biopsy was performed, this time with signs of hemophagocytosis as well as strange inclusion bodies in the macrophages thought to be debris from phagocytosed cells - or unicellular parasites.
Travelling history revealed a trip to Spain 6 months prior to onset of her symptoms. PCR of her bone marrow was positive for Leishmania infantum.

She was started on AmBisome and quickly improved in both her VL and HLH.

Key Learning Points

Symptoms of VL are hidden in the symptoms of HLH, and microscopy of the bone marrow is oftentimes negative in the beginning. If travel history is positive, VL needs to be considered. PCR confirms the diagnosis.

Children in non-endemic areas have in several case reports, as well as in our case, been treated with strong immunomodulation and been close to bone marrow transplant due to progressive HLH as VL was not suspected. Some authors suggest that patients recover from both the infection and secondary HLH on AmBisome alone.

Title of Case(s)

Stuck in the middle with you: An accidental intermediate host

Background

-E. granulosus and E. multilocularis are the two most important Echinococcal species causing infection in humans as accidental intermediate hosts.

-Although these infections are not confined to the tropics, they are rarely acquired in the UK, and are generally considered an imported infection.

-Human infection causes pulmonary and hepatic cysts which require careful excision and a prolonged course of anthelmintics.

Case Presentation Summary

A five-year old Romanian girl, who moved to the UK 3 months earlier, presented febrile in status epilepticus. She has a background of developmental delay and suspected Dravet’s. A chest-xray showed bilateral pulmonary opacifications, so was initially treated for a bacterial pneumonia. A CT chest showed bilateral thick-walled cysts with peribronchial infiltration. Pulmonary tuberculosis was part of the differential. She had no known TB contacts and was unvaccinated, including BCG. Her parents stated that a chest-xray done 18 months previously was normal.

A right-sided chest drain was placed – 80mls of clear aspirate drained. Bronchoscopy and lavage was performed 2 days later. Gastric washings, BAL and pleural samples, and TB ELISpot were negative. A repeat CT chest now identified a possible liver cyst; 2 hepatic cysts were then confirmed on ultrasound. Hydatid serology was negative, however the pleural fluid demonstrated visible protoscolices consistent with E. granulosis. Praziquantel was commenced and she awaits surgical resection, keeping her drain in situ until then.

Key Learning Points

-Different serologic assays have different sensitivities; ELISA being the most sensitive and specific.

-Serologic tests are more sensitive in hepatic disease, infection with viable cysts and E. multilocularis.

-If serology is negative, percutaneous aspiration may be required to confirm the diagnosis.

-Risk of complications such as anaphylaxis and seeding, although low, can be reduced with coverage with praziquantel or albendazole.

Title of Case(s)

Dyspnea after playing in the water canal

Background

Melioidosis, caused by the soil saprophyte Burkholderia pseudomallei, is endemic in South‑East Asia and Northern Australia. The vast majority of infections are asymptomatic; however, in the susceptible host, the infection can result in overwhelming sepsis and death.

Case Presentation Summary

A 6-year-old boy presented with high fever, cough, dyspnea, and weakness for 3 days without vomiting, diarrhea or headache but decreased in appetite and activities. Mum gave only paracetamol at home. His parents are farmers and he had a history of playing in the water canal 2 days before the symptoms started without associated choking or drowning. He was previously healthy and fully vaccinated.

On examination, he had a fever and looked unwell with mild subcostal retraction and crackles on the left side. The cardiovascular exam was normal except tachycardia. An abdominal examination revealed 3cm hepatomegaly.

His initial lab revealed WBC:12x 10⁹ , ANC:5.4x10⁹ , Hemoglobin:88g/L, Platelet:523x10⁹ , Na:138mmol/L, K:3.4mmol/L, CRP:41mg/dL, ALT:32U/L, AST:79U/L, Negative Malaria, CXR showed left upper lobe density.

Initially, he was diagnosed with bacterial pneumonia, treated with ceftriaxone. After 3 days of treatment, his clinical condition became worse. He developed agitation, dyspnea worsened and high fever continued. He was transferred to PICU. The second septic screening was done and revealed increasing of CRP to 174mg/dL and worsen chest X-Ray. His blood culture reported positive Burkhorderia Pseudomallei. Antibiotic therapy was changed to Meropenem immediately. He developed shock unresponsive to several times IVF bolus and severe dyspnea. He was intubated and given inotrope infusion. Unfortunately, he died 1 day later in PICU.

Key Learning Points

Melioidosis commonly causes local infection with associated high mortality in bacteremia, it usually the following exposure to contaminated water in the endemic area. In a child with prolonged fever, a history of water exposure and clinical toxicity Melioidosis must be considered. Delayed diagnosis and management can result in poor prognosis.