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A Case of Focal Segmental Glomerulosclerosis in a Young Girl with a Very Low Birth Weight
Yasuyo Kashiwagi1, Kazushi Agata1, Gaku Yamanaka1
1Department of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, Japan.
Insights
Low birth weight (LBW) and hyperuricemia may contribute to chronic kidney disease (CKD) development in adulthood. Understanding birth history, including very low birth weight, is crucial for diagnosing noncommunicable diseases like focal segmental glomerulosclerosis (FSGS).
Area of Science:
- Nephrology
- Pediatrics
- Public Health
Background:
- Japan has a high prevalence of low birth weight (LBW), estimated at 10%, potentially increasing adult noncommunicable diseases.
- LBW is a recognized risk factor for adult focal segmental glomerulosclerosis (FSGS), but clinical and pathological characteristics remain under-evaluated.
Observation:
- A 13-year-old girl with very low birth weight (630g, 23 weeks gestation) presented with proteinuria and stage 2 chronic kidney disease (CKD).
- The patient and her family members exhibited hyperuricemia, suggesting a genetic predisposition.
- Renal biopsy confirmed focal segmental glomerulosclerosis (FSGS).
Findings:
- Despite treatment with an angiotensin receptor blocker, the patient maintained stage 2 CKD, indicating persistent kidney damage.
- The case suggests a two-hit model: initial insult from LBW followed by hyperuricemia exacerbating kidney disease progression.
- Nephrologists often overlook birth history, which is critical for diagnosing conditions like FSGS.
Implications:
- Collecting detailed birth history, including preterm birth and LBW, is vital for diagnosing adult-onset kidney diseases.
- This highlights the importance of considering LBW and hyperuricemia as contributing factors to CKD in clinical practice.
- Routine school urinary screening, as practiced in Japan, can aid in early detection of kidney issues, but comprehensive patient history is essential globally.
Abstract:
In Japan, the prevalence of low birth weight (LBW) has been estimated to be approximately 10%, which is the highest among developed countries. This high prevalence might affect the occurrence of LBW-associated diseases in the adult population of Japan. LBW has been recognized as a contributing factor to post-adaptive focal segmental glomerulosclerosis (FSGS) in adulthood; however, few reports to date have evaluated the clinical and pathological characteristics of post-adaptive FSGS. A 13-year-old girl was referred to our hospital owing to mild proteinuria, which was detected at a school urinary screening. She was born at a gestational age of 23 weeks, with a very LBW of 630 g. Dipstick urinalysis revealed grade (2+) proteinuria. Her serum creatinine level was 1.02 mg/dL, and she was diagnosed as having stage 2 chronic kidney disease (CKD). Her serum uric acid level was 7 mg/dL. Furthermore, her mother and 16-year old brother had hyperuricemia. A percutaneous renal biopsy leads to a diagnosis of FSGS. After 3 years of treatment with an angiotensin receptor blocker, her proteinuria decreased. However, her serum creatinine level was 1.07 mg/dL, and she still had stage 2 CKD. We considered that in this patient, the first hit was her LBW, and the second hit was hyperuricemia. The second hit might be associated with the development of CKD. The birth history of patients is not usually confirmed by nephrologists. Our case demonstrates that obtaining information regarding the preterm birth and LBW of patients is important in the diagnosis of noncommunicable diseases because school urinary screening is not routinely performed in countries other than Japan.
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