Mother to Child Transmission of Infectious Hemolytic Uremic Syndrome: an Intriguing Case
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Asian Journal of Medical Case Reports 2(1): 5-8, 2020; Article no.AJMCR.360 Mother to Child Transmission of Infectious Hemolytic Uremic Syndrome: An Intriguing Case Suksham Jain1, Anshu Palta2 and Anam Siddiqui1* 1Department of Neonatology, Government Medical College and Hospital, Chandigarh, India. 2Department of Pathology, Government Medical College and Hospital, Chandigarh, India. Author’s contribution This work was carried out in collaboration among all authors. Authors AS and SJ diagnosed the case. Author AP helped in pathological diagnosis and correlation. Authors AS and SJ wrote manuscript. Author AP contributed in finalizing the manuscript. All three authors agreed to final manuscript. Received 16 January 2021 Case Report Accepted 22 March 2021 Published 23 March 2021 ABSTRACT Hemolytic uremic syndrome (HUS) is one of the common causes of acute renal failure in children, but it is rarely encountered in newborns. It comprises of a triad of microangiopathic hemolytic anemia, non-immune thrombocytopenia and renal failure. Neonatal cases of HUS predominantly belong to the atypical category, resulting from abnormalities of the complement pathway or cobalamin C disorders. However, HUS cases due to infection have rarely been reported in newborns. For infection associated HUS e.g., due to Shiga toxin producing E.coli (STEC), presence of diarrhea is not a must and some cases associated with urinary tract infection (UTI) have also been seen. We present a case of a female newborn who developed HUS by acquiring the infection from her mother who had UTI associated HUS. Keywords: Hemolytic Uremic Syndrome (HUS); neonate; vertical transmission; Urinary Tract Infection (UTI). 1. INTRODUCTION cases of HUS due to STEC have been reported [2,3]. In cases due to infection by Shiga toxin– Hemolytic uremic syndrome (HUS) comprises of producing Escherichia coli (STEC), diarrhea is a triad of microangiopathic hemolytic anemia, common but not a must and some cases non-immune thrombocytopenia and renal failure associated with urinary tract infection (UTI) [1]. Neonatal cases of HUS predominantly have also been seen [4,5]. We present a belong to the atypical category resulting from case of a female newborn who developed abnormalities of the complement pathway or HUS by acquiring the infection from her cobalamin C disorders. Nevertheless, neonatal mother. _____________________________________________________________________________________________________ *Corresponding author: Email: [email protected]; Jain et al.; AJMCR, 2(1): 5-8, 2020; Article no.AJMCR.360 2. CASE PRESENTATION was 2 kg at admission. She was managed for severe sepsis with shock, dehydration, and We present a case of a female neonate born to neonatal jaundice, hence started on intravenous primigravida mother at term gestation via normal (IV) fluids, IV meropenem and vancomycin, nasal vaginal delivery with an APGAR score of 7,8 and CPAP, inotropes, and double surface birth weight of 2.4 kg. Baby was started on phototherapy. Lab investigations were breastfeed and passed urine and meconium on suggestive of nonimmune hemolytic anemia with the first day of life. She was apparently well until thrombocytopenia and renal failure [Table 1]. day four of life when her mother noticed Coagulation profile was normal. She received yellowish discoloration of her skin and eyes. Next fluid correction for hypernatremia and antibiotics day, she developed vesicular rash involving were adjusted according to renal dose flexure areas of axilla and groin and partly upper modification. By eighth day of life shock and back and lower abdomen. Subsequently, lesions respiratory distress were passive, sclerema became bluish red and larger in size and baby decreased, icterus subsided, and she was was referred to our center [Fig. 1]. started on orogastric feeds. She passed urine adequately. There was no further evolution of lesions. Dermatology consultation was done, 2.1 Clinical Images of the Newborn Tzanck smear, Gram stain and KOH stain were negative. The urine examination revealed mild At admission, she had cold peripheries with poor albuminuria with no evidence of UTI. Sequential peripheral pulses and respiratory rate of 72 blood cultures were sterile. Her mother was breaths per minute with intercostal retractions. investigated and managed as UTI associated She also had icterus and sclerema. Her weight HUS [Table 1]. Fig. 1. Clinical image of the newborn showing hemorrhagic skin lesions of HUS (solid arrows) Table 1. Investigations of the neonate and her mother Parameter Baby Mother Hemoglobin (g/L) 133 90 Platelet count (x 109/L) 15 58 Total leucocyte count (x 109/L) 15.8 18.1 Differential count (N/L/M/E) 51/38/6/5 78/12/8/2 Peripheral blood film Schistocytes+ Schistocytes+ Reticulocytes (%) 0.40 1.09 Direct Coomb’s Test Negative Serum sodium (mmol/l) 168 130.1 Serum potassium (mmol/l) 6.2 2.9 Urea (mmol/L) 42.45 8.49 Creatinine (umol/L) 176.9 196.4 Total serum bilirubin (umol/L) 256.5 Urine pus cells (per hpf) 0‒1 12‒15 Urine RBC (per hpf) Nil Nil Urine albumin 1+ Nil ANA Negative Culture (urine) E. coli Culture (blood) Sterile Abbreviations: N/L/M/E: neutrophil, lymphocyte, monocyte, eosinophil, hpf: high power field, ANA: anti-nuclear antibody 6 Jain et al.; AJMCR, 2(1): 5-8, 2020; Article no.AJMCR.360 The clinical presentation of the neonate complied ANA had 98.1% specificity and 46.9% sensitivity with the diagnosis of infectious HUS with no [8]. For this reason, ANA was done for the obvious diarrhea or UTI and responded well to mother that came out to be negative. Non- hydration and supportive therapy. Repeat immune thrombocytopenia in association with investigations showed normalization of the microangiopathic hemolytic anemia can also hematological and renal parameters and lesions occur in Kasabach Merritt syndrome and renal gradually underwent scabbing and subsided vein thrombosis. There were no indicators of without any residual hyperpigmentation. The these possibilities in the index case. Since, baby was discharged on day 16 of life on pleomorphic cutaneous manifestations are breastfeed and supplements with stable vitals, known in neonatal hemophagocytic histiocytosis, weight 2.4 kg and normal investigations. this possibility was also kept as a differential [9]. Although the patient had hypothermia and rash 3. DISCUSSION with bi-cytopenia, there was no organomegaly and coagulation profile was normal, hence more In the reported case, the patient presented with in favor of HUS. Infection associated HUS does anemia and jaundice with schistocytes on not require plasma exchange or infusion and peripheral blood film with a negative Coomb’s most of the cases respond well to hydration and test suggesting microangiopathic nonimmune symptomatic therapy [10]. In the reported case hemolytic anemia. Also, there was evidence of also, the newborn was managed symptomatically thrombocytopenia and acute renal insufficiency. with major stress upon hemodynamic status and This clinical picture very well fitted to the triad of sepsis. Without requiring any blood product, the HUS and led us to approach the patient in this baby recovered and went home in a stable direction. Serum haptoglobin and lactate condition with subsiding skin lesions. dehydrogenase levels are used as supportive tools in diagnosing intravascular hemolysis, but 4. CONCLUSION they are unreliable in a newborn due to inherent hepatic immaturity [6]. Similar triad in the mother Although rare, the possibility of HUS must be along with history of active UTI supported the kept in differentials for a newborn presenting with diagnosis of infection-associated HUS. Also, as a the classic triad of TMA. Atypical HUS is more plausible explanation, the mother had transmitted common in newborns, but infection- associated infection vertically to the neonate leading to HUS HUS can also be seen. STEC can cause in the baby. Although, the newborn did not have diarrhea as well as UTI. Vertical transmission of any evidence of diarrhea or UTI and presented STEC from mother to child is possible. as sepsis, this does not exclude the possibility of HUS. Her coagulation profile was normal, CONSENT ruling out disseminated intravascular coagulation due to underlying sepsis. Thrombotic Written informed consent was obtained from the thrombocytopenic purpura (TTP) can also have a parents for publication of the information and similar presentation with thrombocytopenia, images in this case report. hyperbilirubinemia, increased creatinine, hemolysis etc [7]. Notably, TTP is characterized ETHICAL APPROVAL by the triad of thrombotic microangiopathy with predominantly neurological features, as opposed As per international standard or university to predominant renal impairment in HUS. standard written ethical approval has been Congenital TTP is attributed to an inherent collected and preserved by the author(s). deficiency of vWF-cleaving metalloprotease (ADAMTS-13). However, measurement of ACKNOWLEDGEMENT ADAMTS-13 in newborns is highly laboratory- dependent and is a costly investigation. 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