Dr.Sree Lekshmy.S, Dr.Sajitha Nair, Dr.Sindhu, Dr.Sreya,DrC Jayakumar
Three month old female first child of non consanguineous couple ,bottle fed, developed high grade fever, multiple episodes of loose watery stools for 4 days and reduced activity,poor feeding of 2 days . As ba had severe dehydration, acidosis ,bluishness of tounge and features of sepsis (CRP was 203mg/L and procal was 95) ba was referred to AIMS
In view of central cyanosis and abnormal colour of blood, Methemoglobin level was sent and was found to be high (18.7%). Repeat value was also high (17.1%).
ECHO done was normal. Hence the child was started on Vitamin C and was given 2 doses of methylene blue. Due to persistence of loose stools, feeds were changed to lactose free formula(Isomil)
Child was also given one PRBC transfusion
i/v/o low Hb (5.9gm/dl).
Differentials considered:
Acquired Methemoglobinemia
Hemoglobinopathies
Intoxication
Sepsis
At admission to PICU, child was conscious,afebrile, sick looking with stable vitals. There were no features of dehydration. General examination showed some dysmorphic features (Upslanting eyes, pointed nose, prominent, low set with FTT. There was pallor and bluishness of the tongue
There was no jaundice,lymphadenopathy or organomegaly. AF was at level. Systemic examination was normal. VBG showed compensated metabolic acidosis. (pH-7.455, pCO2-15.8, p02-151, MetHb- 1.5, Lac-0.8, HCO3-10.9). CBC showmed anemia, thrombocytopenia and normal WBC counts with lymphocytic predominance (TC-8.80K/uL, N-22.1%, L-56.7%, Hb- 8.8g/dl and Plt- 95K/uL) and CRP of 80mg/L. Peripheral smear showed microcytic hypochromic anemia with eosinophilia and thrombocytopenia. LFT showed S.albumin of 2.9g/dl with normal bilirubin and enzymes. Blood and urine for toxicology analysis was negative for any drugs or toxins. Metabolic acidosis was corrected as per protocol. After sending relevant cultures, child was continued on Inj Cefotaxim, Inj Amikacin, Vitamin C and other supportive measures. Rapid gastroenteritis Multiplex test- Qualitative Real-Time PCR was negative. Stool/blood cultures were negative. In view of persistent loose stools, positive occult blood and reducing substance in stools and methemoglobinemia, a possibility of CMPA was considered and was started on hypoallergenic formula(Neocate). Sigmoidoscopy and HPE done was essentially normal. The ba improved symptomatically.Loose stools subsided and Methemoglobin levels remained normal on serial monitoring. She needed 1 PRBC transfusion during hospital stay. She was shifted out of PICU 3rd day of admission. Neurosonogram was normal. Reports of EEG, TMS, GCMS and WES done outside were followed up and were reported as normal. USG Abdomen showed cholelithiasis with no features of cholecystitis, and Ursodeoxycholic acid (Udiliv)was added at a dose of 15mg /kg/day
At discharge the ba is afebrile, tolerating neocate feeds pallada well. She passes stools normally once a day. Weight at discharge is 3.920kg and VBG is pH-7.381, pCO2-37.1, p02-46.8, Methb-1.4, Lac-1.5, HCO3-21.5.
Parents have been counselled about the issues in the ba,need to continue neocate feeds pallada and medications as advised.
Discussion:
Cow’s milk protein allergy (CMPA) is not a rare condition seen in young children, particularly in the first year of life. Incidence is 2% to 7.5%.
CMPA is classified into immunoglobulin E (IgE)- or non-IgE-mediated reactions.
Non-IgE can be acute or chronic, it includes a range of symptoms predominantly affecting the gastrointestinal system, with varying severity.
Chronic forms of food protein induced enterocolitis syndrome (FPIEs) occur mainly in children under four months of age after repeated ingestion of cow’s milk protein (CMP), characterized intermittent vomiting, chronic diarrhea, and weight loss. Blood tests may show anemia, hypoalbuminemia, thrombocytosis, leukocytosis with left shift and eosinophilia, dehydration, metabolic acidosis, or methemoglobinemia. MTreatment consists of the elimination of CMP from the diet.
Methemoglobin is an altered state of hemoglobin in which the heme iron is oxidized from the ferrous (Fe2+) to the ferric (Fe3+) form. As a result, methemoglobin is insufficient for carrying oxygen, leading to cyanosis. The blood concentration of methemoglobin is normally between 0% and 2%.
There is no exact cause for methemoglobinemia in FPIES,but result in severe intestinal inflammation and decreased catalase activity, resulting in increased intestinal nitrites and increased heme molecule oxidation, which contributes to methemoglobinemia
Treatment with methylene blue should be considered when the concentration rises above 20% in symptomatic patients and 30% in asymptomatic patients, although the decision to treat may be made at lower concentrations if symptoms persist or are severe.