Acute kidney injury requiring renal replacement therapy due to Clostridioides difficile infection in a 15-year-old boy

Acute gastroenteritis is commonly seen in pediatric clinical practice. It is a largely self-limited disease with a benign course. We present a case of teenager with gastroenteritis resulting in severe acute kidney injury. The decline in glomerular filtration was so significant that renal replacement therapy had to be initiated. We had to continue in intermitent hemodialysis for seven days until sufficient improvement in renal function. Clostridioides difficile was identified as a cause of vomiting, bloody diarrhea and subsequent dehydration. To our knowledge, this is the first reported case of C. difficile-associated diarrhea accompanied by acute kidney injury requiring renal replacement therapy in a child.

Juxtaglomerular cell tumor--a rare cause of secondary hypertension.

Secondary hypertension (SH) is much more common in children than in adults. We report a 17-year-old girl with severe hypertension, hypokalemia and metabolic alkalosis. Because of these findings, primary or secondary hyperaldosteronism was suspected. Her initial treatment with spironolactone and ACE inhibitor was unsuccessful. With consideration of high plasma renin activity, the renal computed tomography angiography was performed and showed tumor mass in the left kidney. An uncomplicated partial left nephrectomy was performed. Histopathological examination and electron microscopy showed typical features of juxtaglomerular cell tumor (JCT). Imunohistochemistry of tumor was positive for CD34 and CD117 and this finding is effective in the diagnosis of JCT if immunostain for renin is unavailable. After the resection of JCT, the patient's blood pressure and hypokalemia returned to normal range. JCT is a rare renal neoplasm and an unusual cause of SH in children or adolescents (Fig. 2, Ref. 12).

Acute hyperammonaemic encephalopathy in a female newborn caused by a novel, de novo mutation in the ornithine transcarbamylase gene.

UNLABELLED: A full-term female offspring of a first and uneventful pregnancy presented at 60 h of life with irritability, tachypnea and respiratory alkalosis progressing to deep coma with clinically dominant circulatory failure, tachycardia and hypotension. Diagnosis of ornithine transcarbamylase (OTC) deficiency was made on the basis of hyperammonaemia, hypocitrullinaemia and extreme hyperexcretion of orotic acid. The baby was treated with peritoneal dialysis, arginine hydrochloride and adequate energy supply. DNA analysis revealed an as of yet unidentified missense mutation in the 6th exon of the OTC gene, resulting in a change of lysine to glutamine at position 210 (K210Q). Her parents were not found to carry this mutation, implying that this mutation may have occurred either de novo in the patient or in a parental germ cell. CONCLUSION: An acute neonatal form of OTC deficiency should be considered in the differential diagnosis of coma in female newborns.

Secondary pseudohypoaldosteronism in an infant with pyelonephritis.

BACKGROUND: Urinary tract infections and/or urinary tract anomalies are very frequent among children. Especially in newborns/infants they represent additional factors in the development of secondary pseudohypoaldosteronism. CASE HISTORY: We present an 8-week-old infant who developed hyponatremia and hyperkalemia secondary to acute pyelonephritis. The boy presented with non-specific signs, including poor appetite, lethargy, and hypotonia. An extended evaluation led to the diagnosis of pseudohypoaldosteronism (PHA). PHA was transient and during therapy of pyelonephritis all of abnormal laboratory parameters returned to normal. The patient had vesico-ureteric reflux grade IV. CONCLUSION: Secondary/transient/reversible PHA occurs in patients with immature renal tubular responsiveness to aldosterone due to infancy when they have urinary tract anomalies and/or urinary tract infection. (Tab. 1, Ref. 7.)

The analysis of the risks for the development of tumour lysis syndrome in children.

BACKGROUND: Acute renal failure (ARF) during the course of cytostatic therapy is a serious complication. ARF can be isolated or became as component of tumour lysis syndrome (TLS). TLS comprises a number of metabolic abnormalities (hyperuricemia, hyperphosphatemia, hyperkalemia, azotemia and hypocalcemia) which are associated with lymphoproliferative malignancies following spontaneous or chemotherapy-induced cytolysis. There exist probably no clear prediction for the development of TLS that could enable early detection of manifestation of this severe condition. SUBJECTIVE: Conventional management with aggressive hydration, alkalization of the urine, administration of allopurinol, and the slow introduction of chemotherapy is often unable to prevent metabolic instability and ARF. Recent studies define a subgroup of patients at higher risk of renal failure during induction chemotherapy. ARF was encountered during initial therapy of patients with a lactate dehydrogenase (LDH) index greater than 3.3. METHODS AND MATERIAL: A retrospective analysis of 10 children (3 girls, 7 boys, average age 9.7 years) with LDII index greater than 3.3 has been done. All children were treated for lymphoproliferative malignancy with conventional preventive measures. RESULTS: Three children needed haemodialysis--2 boys had fully expressed TLS with ARF shortly after starting chemotherapy, in 1 boy the dialysis was indicated because of extreme hyperuricemia and high creatinine level presented before chemotherapy. We consider that LDH index is not specific criterium for prediction of TLS. In conclusion, our cases demonstrate the pathophysiologic spectrum of ARF in TLS between hyperuricemia and hyperphosphatemia. CONCLUSION: The LDH index, urine output, and hyperphosphatemia could be used to identify those paediatric patients who would benefit from the prospective use some of extracorporeal elimination methods. Further investigation of this techniques in a larger number of patients is warranted. (Tab. 5, Ref. 12.)

Is it possible to influence the mortality in children with hemolytic uremic syndrome?

THE CURRENT STATE: Hemolytic uremic syndrome (HUS) is the most frequent cause of acute renal failure in children. In our geographic conditions D(+)HUS prevails, in its etiology E. coli O157:H7 is represented most. In the course of HUS there can occur extrarenal damage to some organs, the manifestation of multiple organ failure is then possible. Delayed diagnosis of HUS, its complicated course and therapeutical strategy influence greatly the mortality rate of affected children. SUBJECTIVE: The optimal therapeutical procedure was elaborated on the base of evaluating the causes of death in children with HUS, and, simultaneously, there were revealed further reserves that can decrease the mortality rate. METHODS AND MATERIAL: A retrospective analysis of mortality within 1982-2000 was carried out in one of three centres dealing with the therapy for HUS in children in the Czech Republic. The total number of HUS children was 69 (40 girls, 29 boys, mean age 4.5 years). 9 patients out of the analyzed group died. RESULTS: Out of 9 children (4 girls, 5 boys) died, D(+)HUS was present in 7 (77.8%, 4 girls, 3 boys, mean age 2.1 years), D(-)HUS occurred in 2 subjects (22.2%, boys, mean age 7.5 years). Dialysis therapy was needed in all 9 children (peritoneal dialysis and/or hemodialysis). The mortality rate in our group of children was 13%. An autopsy carried out in dead children showed dominant severe affection of kidneys/brain/heart. CONCLUSION: In spite of marked decrease in the number of HUS children died particularly in developed countries, permanent attention must be paid to this disease. Besides early diagnosis, corresponding therapy is necessary. That should be performed at a specialized centre. The elaborated algorithm can be used in the therapy for HUS. This is one of the ways for further decrease of both mortality and chronic morbidity in children suffering from HUS. (Tab. 3, Fig. 1, Ref. 15.).

[Diagnosis of acute pyelonephritis in childhood: comparison of ultrasonographic examination and renal scintigraphy using 99mTc DMSA]. / Diagnostika akutní pyelonefritidy v detském veku: srovnání ultrasonografického vysetrení a scintigrafie ledvin pomocí 99mTc DMSA.

THE CURRENT STATE: Infections of the urinary tract are a frequent paediatric problem. Their treatment requires to assess the localisation of infection within the uropoetic tract. Especially the acute pyelonephritis (AP) can lead to irreversible changes within the renal parenchyma and alteration of renal functions. Some imaging examinations can help in the assessment of the diagnosis of AP and contribute to appropriate therapy. SUBJECTIVE: The analysis of results gained from imaging examinations in children with AP was based on the comparison of used techniques and the assessment of their exploitation for the diagnosis of AP. METHODS AND MATERIAL: 38 children (17 girls and 21 boys at average age of 11.6 years) with clinical criteria of AP were subdued to ultrasonographic examination with B record (US-B) and ultrasonographic examination with energetic Doppler (US-D). Both methods were compared with the findings gained by use of renal scintigraphy 99mTc DMSA. RESULTS: The positive finding of AP was proved in 36 children (94.7%). The results of US-B proved AP in 14 children (36.8%) of the whole group of patients, i.e. in 38.8% with positive DMSA finding. US-D changes indicated AP in 15 children (39.4%) of the whole group, i.e. in 41.6% with positive DMSA finding. The sensitivity of both methods US-B and US-D appear to be low for the verification of changes in renal parenchyma in AP. CONCLUSION: Despite its advantages and availability, the gain from ultrasonographic examination in children suffering from AP is small. Clinical and laboratory criteria are fully sufficient for AP verification in common clinical practice. DMSA scintigraphy is a method of choice in complicated cases of AP. (Tab. 1, Ref. 17.)

[Screening for alpha 1-antitrypsin deficiency in neonates]. / Screening deficitu alfa-1-antitrypsinu v novorozeneckém veku.

In a two-year investigation 113,274 children were screened for alpha-1-antitrypsin deficiency. An original and cheap method was used. In children with an alpha-1-antitrypsin values lower than 1.5 g/l the phenotype was assessed. In 120 neonates alpha-1-antitrypsin was assessed by screening and also quantitatively. The physiological range of alpha-1-antitrypsin for neonates is: 1.4-3.32 g/l. A low incidence of alpha-1-antitrypsin in the Czechoslovak population, as compared with investigations abroad, was revealed. The authors discuss the possibility to extend screening from the clinical, ethical and economic aspect to the entire republic.

[Cardiorespiratory fitness in children with type I diabetes mellitus]. / Kardiorespiracní funkcní zdatnost detí s diabetes mellitus I. typu.

The authors compared indicators of cardiorespiratory functions of 13 boys and 14 girls with insulin-dependent diabetes with the same parameters in a group of their healthy siblings and with mean values of the Czechoslovak population. No significant differences were found. Practically identical values of the decline of bases in the blood stream of healthy and sick children after a load suggest the ability of diabetics to compensate acidosis after a load. From the investigation it is apparent that in child diabetics in the prepubertal period the possibility of developing physical fitness is preserved. The general higher blood level in diabetics after a short intensive load declined, while it rose in siblings. Parameters of the internal environment differed greatly between individual subjects.